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A  TEXTBOOK 

OF 

NERVOUS   DISEASES 

FOR  STUDENTS  AND  PRACTISING  PHYSICIANS 
IN    THIRTY    LECTURES 

BY 

ROBERT   BING 

DOZENT    FOR    NEUROLOGY    AT    THE    UNIVERSITY    OF    BASEL 

ONLY    AUTHORIZED   TRANSLATION 

BY 

CHARLES  L.  ALLEN,   M.D.,   Los  Angeles,   Calif. 

WITH   ONE-HUNDRED   AND   ELEVEN  ILLUSTRATIONS  IN   THE   TEXT 


9ooo 


NEW    YORK 

REBMAN    COMPANY 

in.   148  and  145  WEST  86th  STREET 


Copyright,   1915,  by 

REBMAN     COMPANY 

New  Yob  k 

All  Rights  reserved 


I'llISIIl)    IN    AMKltll'A 


Preface 

When  it  was  suggested  by  my  publishers  that  I  should  write  a  text-book 
of  nervous  diseases  adapted  to  the  needs  of  students  and  practical  physicians ; 
in  size  between  the  summary  compendiums  and  outlines  and  the  large  refer- 
ence works  and  handbooks  of  neurology.  I  found  upon  inquiry  among  my 
colleagues  so  general  an  agreement  that  such  a  book  was  desirable,  that  I  no 
longer  hesitated  to  undertake  its  preparation.  When  I  decided  to  present  my 
material  in  the  form  of  lectures,  it  was  because  of  the  recollection  that  in  my 
student  days,  the  reading  of  text-books  in  lecture  form  was  to  my  fellow  stu- 
dents and  myself  like  an  oasis  in  the  midst  of  the  tiresome  work  of  preparing 
for  examinations.  On  the  other  hand,  I  did  not  conceal  from  myself  that  the 
systematic  arrangement  of  the  material,  as  compared  to  its  presentation  in 
lectures,  had  in  general  the  advantage  of  greater  clearness  and  better  adapta- 
tion for  quick  reference.  On  this  account  I  have  endeavored  to  combine  the 
advantages  of  both  methods  of  arrangement,  by  the  introduction  of  titles,  sum- 
maries in  tabular  form,  and  recapitulatory  headings.  I  have  also  laid  great 
stress  upon  a  complete  index. 

In  the  arrangement  of  the  subject  matter,  I  have  subordinated  the  cus- 
tomary topographical  and  pathologico-anatomica]  classification  almost  en- 
tirely to  the  etiological  and  pathologico-physiological,  as  a  glance  at  the  table 
of  contents  will  show:  I  was  so  enabled,  for  example,  by  grouping  together  the 
syphilogcnic  diseases  of  the  whole  central  nervous  system,  through  the  con- 
sideration in  common  of  the  most  varied  disturbances  of  conduction  in  the 
territory  of  the  peripheral  nerves,  by  the  clinical  synthesis  of  the  diverse 
varieties  of  spinal  "Transverse  lesions,"'  by  handling  in  common  the  "Dyskine- 
sias," the  "Dysglandular  Symptom-complexes,"  etc..  to  avoid  much  repeti- 
tion. This  was  all  the  more  necessary,  since  in  spite  of  my  plan  of  limiting 
the  si/e  of  my  text-book,  I  found  it  necessary  to  treat  somewhat  at  length, 
subjects  of  special  Importance  to  the  practicing  physician;  in  fact  in  some 
instances  to  go  more  into  detail  than  has  so  far  been  done  in  the  larger  works: 
so  for  example,  as  to  the  nervous  manifestations  of  arteriosclerosis,  the  treat- 
ment of  tabes,  of  neuritis  and  neuralgia,  the  psychology  of  the  neurasthenic,  etc. 

Further  in  favor  of  this  plan,  was  the  fact  that  with  regard  to  localization; 
by  referring  to  my  "Compendium  of  Topical  Diagnosis  of  Diseases  of  the 
Brain  ami  Spinal  Cord"  from  which  a  few  loans  have  been  obtained,  I   have 

been   enabled   to  treat   the  matter  in   a   manner  very  condensed,  but   adequate   lo 
the  scope  of  this  book. 

With  all  justice  to  my  endeavor  to  presenl  particularly  my  own  experiences 
and  my  own  views,  I  believe  thai  1  have  been  careful  enough  in  imparting  the 


vi  PREFACE 

observations  and  opinions  of  other  authors  to  hope  that  my  book  may  present 
to  the  non-neurologist  a  well-rounded  view  of  our  specialty,  which  unfor- 
tunately still  suffers  in  some  places  from  insufficient  consideration  in  the  clinical 
courses  of  instruction. 

In  this  connection  I  have  refrained  from  forcing  to  the  front  the  views  of 
any  particular  school,  rather  have  I  endeavored  to  exercise  a  certain  eclecti- 
cism, which  is  justly  considered  as  the  scientific  signature  of  our  country, 
in  which  the  German  and  the  Latin  manner  of  thought  and  investigation  blend, 
and  reinforce  one  another  in  harmonious  fashion.  With  regard  to  the  illus- 
trations of  my  book,  it  was  not  my  intention  to  prepare  an  atlas  of  rare  and 
atypical  cases,  but  rather  to  bring  before  the  reader  the  instructive  and  char- 
acteristic of  the  more  important  morphological  anomalies.  Where  my  own 
photographs  and  sketches  have  been  insufficient,  I  have  been  aided  in  the  most 
friendly  manner  by  my  colleagues  who  have  put  their  material  at  my  disposal. 
I  am  indebted  in  the  highest  degree  to  Doctors  Eduard  Hagenbach,  F.  de 
Quervcdn,  B.  Stahelm,  E.  W'uland,  E.  Villiger,  B.  Block,  P.  Knapp,  and 
H.  Iselin  of  Basle,  A'.  Frey  and  E.  Bircher  of  Aarau,  E.  Buppaner  of  Samaden. 
All  the  photomicrographs  were  kindly  prepared  by  my  Assistant  Dr.  Leuen- 
berger  from  preparations  of  my  collection, 

Basle  Robert  Bing. 

In  translating  this  thoroughly  useful,  practical  and  up-to-date  book,  it  has 

been  endeavored  to  present  the  author's  views  as  nearly  in  his  own  language 

as  is  consistent  with  clear  and  readable  English.     An  occasional  foot-note  of 

explanation,  or  of  reference  to  matters  which  have  come  up  since  the  German 

Edition  was  published,  has  been  added.  ..,„.  .^^^ 

r  TRANSLATOR. 


Table  of  Contents 

Lecture  I. — Diseases  of  the  Peripheral  Nerves — A.  Disturbances  of  Conduc- 
tion— 1.    Spinal  Nerves.  Page   1 

Lecture  II. — Peripheral  Nerves  (Cont'd) — 2.  Cranial  Nerves — B.  Neuritis 
and  Polyneuritis.  Page  26 

Lecture  III.- — Peripheral  Nerves   (Cont'd) — C.   Neuralgias — D.   Treatment. 

Page  45 

Lecture  IV. — The  Dyskinesias — A.  Tremor — B.  Fibrillary  Contractions — 
C.  Muscular  Spasms.  1.  Local  Spasms — 2.  Occupation  Spasms — 3.  Myo- 
klonias — L  Tetany.  Page  63 

Lecture  V. — The  Dyskinesias  (Cont'd) — D.  Choreiform  Diseases.  1.  Chorea 
Minor — 2.  Hereditary  Chorea — E.  Athetoses — F.  Paralysis  Agitans — G. 
Myotonia — H.  Congenital  Muscular  Atrophy — I.  Periodic  or  Paroxysmal 
Paralysis — J.  Myasthenia.  Page  80 

Lecture  VI. — Progressive  Muscular  Atrophies — A.  Myopathic  Form — B. 
Neural   Form — C.   Spinal  Form.  Page  97 

Lecture  VII. — Spastic  Spinal  Paralysis — Amyotrophic  Lateral  Sclerosis  and 
Progressive  Bulbar  Paralysis.  Page  111 

Lecture  VIII. — Hereditary  Family  Ataxias — I.  Friedreich's  Disease — II. 
Cerebellar    Heredo     Ataxia — III.     Infantile    Progressive    Hypertrophic 

Neuritis.  Page  123 

Lecture  IX—  Multiple  Sclerosis.  Page  132 

Lecture  N. —  Further  Diffuse  Diseases  of  the  Spinal  Cord — A.  Spinal  Gliosis 

and     Syringomyelia — B.     Hematomyelia — C.    Transverse     Lesions     of    the 
Spinal     Cord — 1.     Genera]     Symptomatology     2.     Diffuse     Myelitis     3. 

Tumors  of  the  Spinal  Cord 1.   Lesions  of  the  Spinal  Cord  due  to    Affec 

tions  of  tin    Vertebral   Column.  I'm,/''  1*8 

I.i'  ii  i;  i    XI.     The  Syphilogenic  Diseases  of  the  Central  Nervous  System 
A.  Tabes  1  )orsalis.  P<i<j<    1  <>  I 

vii 


viii  TABLE    OF    CONTEXTS 

Lecture  XII. — The  Syphilogenic  Diseases,  etc.  (Cont'd) — A.  Tabes  Dorsalis 
{Cont'd).  Page  182 

Lecture  XIII. — The  Syphilogenic  Diseases,  etc.  (Cont'd) — B.  Progressive 
Paralysis  of  the  Insane  (General  Paresis).  Page  200 

Lecture  XIV. — The  Syphilogenic  Diseases,  etc.  (Cont'd) — C.  Syphilogenic 
Combined  System  Diseases — D.  Cerebro-Spinal  Syphilis.  Page  213 

Lecture  XV. — Arteriosclerosis  of  the  Nerve  Centers,  Page  222 

Lecture  XVI. — The  Acute  Infectious  Diseases  of  the  Central  Nervous  Sys- 
tem— A.  "Essential  Infantile  Paralysis'"  (The  "Meine-Medin  Disease") 
(Anterior  Polyomyelitis) — B.  Epidemic  Cerebro-Spinal  Meningitis. 

Page  235 

Lecture  XVII. — Encephalorrhagia  and  Encephalomalacia  (Supplementary) 
Atypical  and  Extracapsular  Hemiplegias.  Page  25(5 

Lecture  XVIII. — Aphasia,  Apraxia  and  Agnosia.  Page  272 

Lecture  XIX. — Tumor  Formations,  Inflammations,  and  Disturbances  of 
Circulation  in  the  Brain  and  its  Membranes — A.  Brain  Tumor — B. 
Brain  Abscess — C.  Purulent  Cerebral  Meningitis — D.  Tuberculous  Menin- 
gitis— E.  Internal  Hemorrhagic  Pachymeningitis — F.  Thrombosis  of  the 
Brain  Sinuses — G.  Non-suppurative  Encephalitis — 1.  Polioencephalitis 
Superior  Hemorrhagica — 2.  Acute  Hemorrhagic  Encephalitis  of  Adults — 
H.  The  Circulatory  Disturbances  of  the  Brain.  P(l9e   288 

Lecture  XX. — Diseases  of  the  Cerebellum — A.  Tumors — B.  Abscesses — C. 
Serous  Meningitis  of  the  Posterior  Fossa  of  the  Skull — D.  Hemorrhages 
and  Softenings — E.  Agenesis  and  Atrophies — F.  Infectious  Diseases. 

Page  306 

Lecture  XXI. — Malformation,  Congenital  and  Early  Acquired  Defective 
Conditions — A.  Hydrocephalus — B.  Cranial  and  Spinal  Ectopics — C. 
Congenital  Nuclear  and  Muscle  Defects.  Page  317 

Lecture  XXII. — Infantile  Spastic  Hemiplegia  and  Diplegia;  Little's  Dis- 
ease; Idiocy.  Page  327 

Lecture  XXIII. — Dysglandular  Symptom-Complexes — I.  Basedow's  Disease. 

Page  345 


TABLE    OF    CONTENTS  ix 

Lecture  XXIV. — Dysglandular  Symptom-Complexes  (Cont'd) — II.  Myxe- 
dema— III.  Adrenal  Insufficiency  and  Addison's  Disease — IV.  Acromegaly 
■ — V.  Other  Dysglandular  Syndromes — Adiposo  Genital  Degeneration — 
Dyspinealism.  Page  358 

Lecture  XXV. — Diseases  of  the  Sympathetic,  Angio-  and  Tropho-Neuroses 
— A.  Diseases  of  the  Sympathetic — B.  Acroparesthesia — C.  Angiospastic 
Dysbasia — D.  Angiospastic  Symmetrical  Gangrene,  Raynaud's  Disease — 
E.  Scleroderma — F.  Neurotic  Dropsy — 1.  Circumscribed  QOdema  of  the 
Skin — 2.  Intermittent  Dropsy  of  the  Joints — G.  Erythromelalgia — H. 
Facial  Hemiatrophy  and  Hemi-hypertrophy — I.  Herpes  Zoster. 

Page  369 

Lecture  XXVI. — Epilepsy.  Page  388 

Lecture  XXVII. — The  Psychoneuroses — A.  Psychoneuroses  and  Neuro- 
pathic Diathesis — B.  Neurasthenia.  Page  403 

Lecture  XXVIII.  —  The  Psychoneuroses  (Cont'd) — B.  Neurasthenia 
(Cont'd).  Page  418 

Lkcttre  XXIX. — The  Psychoneuroses  (Cont'd) — C.  Hysteria.  Page  436 

Lecture  XXX. — Migraine.  Page  454 

Index  to  Authors  Page  463 

General  Index  Page  467 


List  of  Illustrations 

FIO.  PAGE 

1.  Electric  Irritability  in  "Reaction  of  Degeneration"    11 

2.  Myographic  Curves  on  Direct  Stimulation  of  a  Muscle  with  the  Galvanic 

Current     12 

3.  Motor   Points   of  the   Xeck  and  of  the  Trigeminus    1(3 

1.      Motor   Points  of  the   Face     17 

5.  Motor  Points  of  the   Upper  Extremity.  Outer   Side     18 

6.  Motor   Points  of  the   Upper  Extremity,   Inner  Side    18 

7.  Motor   Points   of   the   Lower   Extremity,  Anterior  View    If) 

8.  Motor   Points  of  the   Lower   Extremity,   Posterior   View 20 

9-      Paralysis   of  the  Serratus  on  the  Right  Side ' 21 

10.  Paralysis  of  the  Suprascapular  Nerve  on  the  Right  Side.  Atrophy  of  the 

Supra-  and  Infra-Spinatus 21 

1 1   and  12.      Ulnar  Paralysis  (Trauma),  "Claw-hand" 22 

18.      Ulnar  Paralysis    (Neuritis)    22 

11.  "Wrist-Drop"  in  Paralysis  of  the  Radial  Nerve   22 

1  5.      Traumatic  Paralysis  of  the  Peroneal  Nerve 23 

Hi  and   17-      Areas  of  the  Different  Sensory  Nerve  Trunks  and  Their  Branches  24 

18.      Left-Sided  Oculomotor  Paralysis.     Ptosis    27 

If).      Paralysis  of  the  Internal  Rectus  on  the  Right  Side 28 

20.  The  Sensory  Nerve  Supply  of  the  Head 80 

21.  Bilateral   Abducens   Paralysis    :U 

22.  Left-Sided  Peripheral  Facial  Paralysis 32 

23.  Left-Sided  Peripheral  Facia]  Paralysis,  Upon  Attempt  to  Close  Both  Eyes  34 

2  1.     Diagram  of  the  Different  Categories  of  Fibers  in  the  facial  Nerve 35 

25.  [mage  "I  the  Vocal  Cords  on  Inspiration  38 

26.  Tuberculous   Polyneuritis    12 

27.  Hereditary  family  Essential  Tremor,    Handwriting til 

28.  "Uisus  Sardonicus"  in  Traumatic  Tetanus   (i(i 

29-      Spasmodic  Torticollis    (if) 


xii  LIST    OF    ILLUSTRATIONS 

FIG.  PAGE 

30.  Parathyroid   Tetany    76 

31.  Athetosis     86 

32.  Paralysis  Agitans.     Handwriting 87 

33.  Typical  Attitude  in  Paralysis  Agitans   88 

34.  Rachitic   Myopathy    93 

35.  Alteration  of  the  Muscle  in  Progressive  Muscular  Atrophy    98 

36.  Progressive    Muscular    Dystrophy    100 

37.  Progressive  Muscular  Dystrophy.     (Type  Erb) 102 

38.  Excessive    Clubbing   of   the    Foot   on    Account   of    the    Gastrocnemius    in 

Progressive  Muscular  Atrophy    103 

39.  The  Babinski  Reflex    114 

40.  Alteration  of  the  Spinal  Cord  in  Amyotrophic  Lateral  Sclerosis 117 

41.  Friedreich's   Disease    125 

42.  Alteration  of  the  Spinal  Cord  in  Friedreich's  Disease 126 

43.  Atrophic   Cerebellum  of   a   Patient   Aged    Forty   Years,   with   Hereditary 

Ataxia     130 

44.  Normal  Cerebellum  of  a  Man  Aged  Forty  Years 130 

45.  Multiple    Sclerosis    133 

16.      Multiple  Sclerosis.     Handwriting 135 

47.  Central  Gliosis  Passing  Over  Into  Syringomyelia 144 

48.  Extent  of  the  Anesthesia  in  Transverse  Lesions  at  Different  Levels.  ...  150 

49-  Complete  Severing  of  the  Spinal  Cord  at  the  Level  of  the  Yllth  Cervical 

Segment    151 

50.  The   Brorvn-Sequard  Symptom-Complex    155 

51.  The   Broirn-Sequard   Symptom-Complex    156 

52.  Tracts  which  Degenerate  Upward  and  Downward 158 

53.  Paraplegia,  with  Extreme  Contracture  in  Flexion 161 

54.  The  Different  Categories  of  Fibers  in  the  Posterior  Roots  and  their  Con- 

tinuation in  the  Spinal  Cord   167 

55.  Structure  of  the  Posterior  Columns   168 

56.  Lumbar   Tabes    (Cervical   Region)     169 

57.  Lumbar  Tabes    (Dorsal   Region)     170 

5S.      Lumbar  Tabes    (Lumbar  Region)     171 

59.     Genu  Recurvatum    174 

60  and  61.      Root  Fields  and  Peripheral  Nerve  Areas 176 


LIST    OF    ILLUSTRATIONS  xiii 

FIG.  PAGE 

62.  Typical    "Radicular"    Disturbances    of    Sensibility    in    a    Case    of    Tabes 

Dorsalis     177 

63.  Tabetic   Foot    ' 178 

61.1 

65.  L Exercise  Treatment  in   Tabes   Dorsalis    ]<)S 

66.  J 

67.  Changes  in  the  Spinal  Cord  in  Syphilitic  Spinal  Paralysis 214 

68.  The  Combined  Tabes    215 

69-     Status    Lacunaris    Cerebri     228 

70.  Pes  Equinovarus  as  a  Result  of  Anterior  Poliomyelitis 238 

71.  Paralytic  Pes  Cavus  as  a  Result  of  Anterior  Poliomyelitis 239 

72.  Infantile  Spinal  Paralysis.     Late  Stage    240 

73.  Infantile   Spinal    Paralysis.      So-called    "Hand    Walker,"    etc 241 

74.  The  Blood  Supply  of  the  Spinal  Cord.     Injected   Preparation   (Carmine 

Gelatine)     244 

75.  Acute  Anterior   Poliomyelitis   in   Childhood    245 

76.  Anatomical  Points  for  Guidance  in  Making  a   Lumbar   Puncture    253 

77.  Introduction  of  the  Needle  in  Lumbar  Puncture    251 

78.  Arterial  Supply  of  the  Cerebrum  and  the  Basal  Ganglia 257 

79-      The  Internal  Capsule  and  Corona  Radiata    258 

80.  Visual   Tract  and   Pupillary   Reflex   Paths    2.">9 

81.  Cerebral  Hemiplegia  from  Capsular  Hemorrhage  on  the  Right  Side 260 

82.  The  Cortical   Speech   Centers   and   Their  Connections    274 

83.  Seat  of  a  Cortical   Motor  Aphasia    275 

84.  Papilledema.     Sarcoma  of  the   Left  Cerebellar  Hemisphere   291 

85.  .Motor.    Sensible    and    Sensory    Cortical    Fields     292 

86.  Motor,   Sensible   and   Sensory   Cortical    Fields     298 

87.  Serous   Meningitis   of   the    Posterior    Fossa   of   the   Skull.       Ideal    Sagittal 

Section    314 

.ss  .-ind  89.     Congenital   Hydrocephalus   818 

90.  Superior   Occipital    1  1  vdroecphalocele    ;!21 

91.  Occult  Spina  Bifida  with  Local  Hypertrichosis 322 

92.  Open  Spina   Bifida   (Cervical  Meningocele)    323 

93.  Diplegia  Spastica    Infantilis   :;:;i 

94.  Diplegia   Spastica    Infantilis    


xiv  LIST    OF    ILLUSTRATIONS 

FIG.  PAGE 

95.  Pithecoid   Idiot    336 

96.  Eye-Ground  in  Amaurotic  Idiocy 338 

97.  Microcephalous   Idiot    339 

98.  Mongoloid   Idiot    340 

99.  Alpine    Cretin    342 

100.  Tragic  Look  in  Basedow's  Disease K 3-16 

101.  Basedow's   Disease    347 

102.  Myxoedema     ' 360 

103.  Plan  of  the  Sympathetic  Nervous  System 370 

104.  Homer's  Symptom-Complex    372 

105.  Sclerodactylism,   with   Formation   of   Necroses    378 

106.  Scleroderma   379 

107.  Herpes  Zoster  of  the  Trunk    384 

108.  Herpes  Zoster  of  the  Face 385 

109.  Modelling's    Disease    387 

110.  Typical  Localizations  of  Hysterical  Topalgias   (shaded)   and  Anesthesias 

( Black)     438 

111.  Hysterical  Self-Injury.     Pseudo  Pemphigus 451 


LECTURE  I 

Gentlemen  :  In  this  course  of  lectures  I  will  endeavor  to  guide  you  in 
a  way  differing  somewhat  from  the  customary  one,  through  the  extensive  and 
interesting  subject  of  clinical  neurolog}'.  I  will  not  treat  separately  the  general 
symptomatology  and  diagnosis  of  nervous  diseases,  neither  will  I  precede  the 
account  of  individual  affections  by  a  special  consideration  of  their  pathology  as 
is  customary  in  most  text-books.  It  seems  to  me  more  correct  didactically, 
to  proceed  directly,  without  the  usual  introductory  remarks,  to  the  study  of 
the  clinical  pictures  and  to  describe  the  methods  of  examination  and  the  inter- 
pretation of  the  different  neurological  symptoms  and  syndromes,  as  digressions 
at  the  points  where  they  fit  most  naturally  and  with  least  compulsion,  into  the 
general  presentation  of  the  subject.  The  same  considerations  apply  to  the 
anatomical,  physiological  and  pathological  data,  which  are  indispensable  for  an 
understanding  of  the  clinical  material.  However,  care  will  be  taken  that  the 
treatment  of  these  matters  is  sufficiently  restricted  not  to  interfere  with  the 
clinically  useful  character  of  the  book. 

Since  practical  neurology  is  to  be  treated,  and  above  everything  the  point 
of  view  of  the  non-specialist  is  to  be  borne  in  mind,  rare  affections  are  brought 
forward  and  described  it  is  true,  but  those  diseases  of  the  nervous  system  which 
on  account  of  their  frequency  are  met  with  in  daily  practice  are  more  particu- 
larly considered  and  carefully  described.  We  will  also  lay  great  stress  upon 
questions  of  treatment  and  will  endeavor  to  prevent  occupying  ourselves  so 
far  with  the  "School  Cases"  and  their  great  symptom-complexes,  that  the 
"minor"  neurology  of  office  practice,  an  important  and  thankful  field  for  the 
development  and  exercise  of  professional  ability  and  therapeutic  tact,  is  not 
forced  into  the  background. 


Diseases  of  the  Peripheral  Nerves 

The  affections  of  the  peripheral  nerves  agree  so  generally  in  their  diag- 
nostic relations,  that,  we  can  readily  consider  their  symptoms  from  two  clinical 
points  of  view.  Without  doing'  violence  to  the  facts,  we  will  hence  consider  in 
common  all  disturbances  of  conduction,  whether  of  traumatic,  neuritic  or  neo- 
plastic origin,  and  after  that  will  take  up  peripheral  nervous  irritative  symp- 
toms, which  in  part  have  their  origin  in  neuritic  processes,  in  part  as  neuralgias, 
can  claim  a  certain  clinical   autonomy. 

Considered  on  the  contrary  from  the  standpoint  of  pathology,  the  diseases 
of  the  peripheral  nerves  present  a  great  multiplicity  of  clinical  pictures,  as  many 

1 


2  LECTURE    I 

in  fact  as  there  are  peripheral  nerves,  and  their  recognition  and  treatment  pre- 
supposes intimate  familiarity  with  anatomical  and  physiological  data. 

In  order  therefore  to  adhere  to  our  plan  and  to  avoid  burdening  you  with 
tiresome  repetition  and  unnecessary  details,  I  will  consider  the  material  under 
the  following  divisions : 

1.  The  peripheral  disturbances  of  nerve  conduction. 

a.  In  the  spinal  nerves. 

b.  In  the  cranial  nerves. 

2.  Special  remarks  on  neuritis  and  polyneuritis. 

3.  The  neuralgias. 

4.  The  treatment  of  the  diseases  of  the  peripheral  nerves. 

A.    Disturbances  of  Conduction 

Etiology 

Traumatic  influences  of  various  kinds  play  a  very  important  role  as  start- 
ing points  for  destructive  lesions  of  the  peripheral  nerves.  In  the  first  place 
we  would  mention  wounding  or  severing  of  the  nerve  trunks  as  they  occur  in 
the  extremities  in  stab  or  incised  wounds,  from  penetrating  projectiles,  some- 
times from  the  splinters  of  a  fractured  bone,  etc.  A  single  severe  compression 
if  it  affects  nerves  exposed  in  a  superficial  position  may  also  cause  interruption 
of  conduction,  so  for  example  in  one  of  my  cases  a  musculo-spiral  paralysis  was 
caused  by  the  patient  being  struck  over  the  region  where  this  nerve  passes 
around  the  outside  of  the  humerus,  by  a  falling  boiler.  Most  frequently,  how- 
ever, the  duration  of  the  compression  is  longer.  So,  paralyses  of  the  brachial 
plexus  are  produced  by  the  pressure  of  a  crutch,  by  the  nerve  plexus  being 
caught  under  the  clavicle  in  over-elevation  of  the  arm  in  prolonged  narcoses,  or 
as  a  result  of  dislocations  of  the  shoulder.  Paralyses  of  the  musculo-spiral 
nerve  are  sometimes  produced  in  prisoners  by  too  tightly  tying  their  arms.* 
Peroneal  paralysis  has  been  known  to  arise  in  agricultural  laborers  from  pro- 
longed squatting;  in  fact  falling  asleep  with  the  knees  crossed  has  been  some- 
times known  to  seriously  injure  the  compressed  peroneus.  Other  "sleep- 
paralvses"  are  seen  not  infrequently  in  the  musculo-spiral  and  ulnar  nerves, 
in  persons  who  sleep  at  night,  the  head  upon  the  outstretched  arm,  or  in  those 
who  enjoy  a  noonday  nap  their  elbows  resting  upon  the  table.  On  account  of 
constantly  supporting  the  elbows  against  the  hard  table  edge,  "professional 
paralyses"  of  the  ulnar  nerve  occur  in  glass  workers,  xylographers,  telephone 
operators,  etc. 

A  cervical  rib  can  produce  paralysis  of  the  dilator  pupillas  and  tarsalis 
superior  muscles,  by  pressure  upon  the  sympathetic  nerve  in  the  neck.  Too 
tightly  put  in  plaster,  in  extension  apparatus,  compression  by  growing  bone 
callus  or  by  a  tumor,  the  application  of  the  Esmarch  bandage  in  checking 
hemorrhage,  may,  on  account  of  their  relative  frequency,  be  mentioned   from 

*  This  refers  to  the  habit  of  the  Continental  Police  of  pinioning  the  prisoner's  arms  behind 
his  back.     (Translator.) 


DISEASES    OF    THE    PERIPHERAL    NERVES  3 

an  almost  endless  list  of  causes  of  compression  paralysis.  Finally,  strain  and 
overstretching  of  nerve  trunks  can  cause  a  break  in  conduction,  which,  not  to 
speak  of  obstetrical  paralysis  in  children  forcibly  extracted,  plays  a  part  in 
accident  practice.  For  example,  I  saw  in  a  stableman  a  serratus  paralysis 
produced  by  a  horse  whose  hind  leg  he  was  holding  to  be  shod,  suddenly  pulling 
it  loose;  in  a  plasterer,  median  paralysis  due  to  the  sudden  jerking  out  of 
his  hand  of  a  rope  by  the  fall  of  a  heavy  box  of  plaster  to  which  it  was 
attached,  etc. 

"Neuritic  processes"  represent  the  second  subvariety  of  disturbances  of 
peripheral  nerve  conduction.  Indeed,  the  term  neuritis,  though  sanctioned  bv 
clinical  use,  cannot  stand  a  strictly  scientific  criticism.  Rather  do  we  find  in 
this  group  of  diseases,  not  only  inflammatory,  but  also  primarily  degenerative 
processes,  and  we  can  assure  ourselves  that  even  in  histopathological  relations, 
as  we  will  see  directly,  the  lines  between  these  two  categories  cannot  be  too 
sharply  drawn.  The  list  of  causes  which  have  been  held  responsible  for  neuritis 
is  varied  enough.  Particularly  often  are  there  in  the  history,  statements  about 
exposure  to  cold.  Vague  and  unsatisfactory  as  this  idea  is,  we  cannot  get 
rid  of  it.  To  select  one  example:  in  the  so-called  rheumatic  facial  paralysis, 
in  which,  according  to  the  findings  of  Minkowski,  Dejerine  and  Theohari  and 
others,  there  is  a  neuritic  or  degenerative  process  affecting  particularly  the 
peripheral  portions  of  the  facial  nerve,  Remak  found  in  45%,  I  in  58%,  and 
other  neurologists  in  as  high  as  70%  of  the  cases,  a  history  of  exposure  to  cold, 
which  not  rarely  appeared  to  have  acted  specially  upon  the  affected  side  (a 
draft).  Neuritis  from  overuse  has  undoubtedly  been  demonstrated.  Frank 
Smith  first  described  a  ''Hammer  Palsy"  which  he  saw  develop  in  the  right  arms 
of  smiths  in  the  Sheffield  Iron  Works,  after  excessive  exertion ;  Coistcr  called 
attention  to  an  atrophy  of  the  small  muscles  of  the  hand,  with  pain  in  the 
median  distribution,  which  occurs  in  cigar-makers  and  a  similar  involvement 
has  been  observed  in  milkmaids,  drummers  and  locksmiths;  musculo-spiral 
paralysis  is  found  in  weavers,  ulnar  paralysis  in  oarsmen,  etc.* 

Now,  however,  in  these  neuritides  from  cold  and  from  overfunction,  it  is 
very  much  of  a  question  if  these  factors  are  exclusively  responsible  for  the 
production  of  degenerative  processes  in  the  affected  nerves.  Very  often  further 
inquiry  develops  the  fact  that  the  real  cause  of  the  disease  lies  deeper,  that  for 
example  alcoholism,  disturbance  of  nutrition,  or  infectious  diseases,  have  so 
modified  the  nervous  system  of  the  patient  that  these  accidental  noxious  influ- 
ences can  exert  a  pathogenic  action.  When  on  any  account,  metabolism  in 
nerves  is  so  altered  that  material  for  the  replacement  of  the  constituents  used 

'  These  neuritides  from  overuse  have  been  grouped,  tdgether  with  the  pressure  paralyses  of 
certain  nerves,  in  persons  of  definite  callings  as  "professional  pareses."  To  these  must  he 
added  certain  "atrophies  from  overuse,"  which  probabrj  do  no!  depend  upon  neuritic  processes, 
inii  .Hi-  ni  myopathic  nature,  since  tin-  affected  muscles  do  no!  correspond  in  their  grouping  to 
those  supplied  by  a  definite  nerve,  the  electric  examination  gives  no  indication  of  the  neuro- 
genic origin  of  the  symptoms,  neither  are  there  pain,  sensitiveness  to  pressure  in   the  nerve,  nor 

sensory  disturbances.    For  example,  in  glass-blowers,  an  atrophj  of  the  muscles  of  the  cheeks 

has  been  observed,  .nut  I  saw  in  ;i  si swhal  anemic  dentist  an  atrophy  of  the  deltoid  muscle, 

apparently  from  constantly  holding  up  the  electric  drill. 


4  LECTURE    I 

up  in  functionating,  is  available  in  less  quantity  than  normal,  as  Edinger  has 
shown,  the  addition  of  noxious  influences  in  themselves  slight,  can  so  disturb 
the  already  unstable  trophic  equilibrium,  that  clinical  and  anatomical  manifes- 
tations of  disease  are  produced. 

On  the  other  hand,  in  the  cases  in  which,  in  a  previously  healthy  person, 
after  severe  chilling,  a  neuritis  develops,  as  in  other  rheumatic  troubles,  the 
supposition  that  the  injurious  influence  of  the  refrigeration  through  reduction 
of  the  general  and  local  resistance  of  the  organism  has  opened  the  door  to  some 
sort  of  infectious  process,  and  has  only  indirectly  acted  in  producing  organic 
lesion,  is  altogether  plausible. 

In  toxic  and  infectious  neuritides,  as  well  as  in  those  occurring  in  the 
course  of  general  nutritional  and  metabolic  disturbances,  we  can  base  our  views 
as  to  etiology  upon  a  firmer  foundation.  Of  all  exogenic  poisons  alcohol  stands 
at  the  head,  indeed  not  in  the  sense  that  a  single  alcoholic  intoxication,  even 
the  most  intense,  is  able  to  set  up  neuritic  processes,  but  rather  that  chronic 
alcoholism  in  part  causes  neuritides  by  itself  alone,  and  in  part  produces  a 
very  decided  predisposition  to  them,  on  which  account,  for  example  among  the 
victims  of  "professional  pareses,"  drinkers  are  present  in  very  high  percentage. 
Oppenhcim  hence  speaks  with  justice  of  "toxico-professional"  paralyses. 
Chronic  lead  poisoning  also  plays  an  important  role ;  type-setters,  painters, 
glaziers,  type-founders,  and  polishers,  etc.,  are,  as  is  well  known,  particularly 
exposed,  and  these  callings  also  furnish  their  contingent  to  the  toxico-pro- 
fessional cases.  Contrary  to  alcohol  and  lead,  arsenic  can  set  up  neuritides 
after  acute  poisoning.  (I  saw  a  case  after  an  attempt  at  suicide.)  Other 
poisons  which  may  be  mentioned  here  are,  ethereal  oils,  carbon  bisulphide,  car- 
bonic oxide,  dinitro-benzol,  anilin,  phosphorus,  mercury,  copper  and  silver. 
In  one  of  my  cases,  in  a  worker  at  a  factory  for  chemicals,  combined  intoxica- 
tion, with  the  vapors  of  bromide  of  methyl  and  methyl  alcohol  had  preceded  the 
outbreak  of  the  polyneuritic  symptoms.  Among  infectious  diseases  which  can 
set  up  neuritis  are  to  be  mentioned  typhoid  fever,  influenza,  pneumonia,  erysip- 
elas, diphtheria,  gonorrhea,  septicemia  (puerperal),  malaria,  relapsing  fever, 
syphilis,  amebic  dysentery,  and  tuberculosis.  In  these,  the  respective  bacteria 
are  not  to  be  found  in  the  affected  nerves,  so  that  only  toxine  action  can  come 
into  question.  Lepra  alone  produces  multiple  foci  in  the  peripheral  nervous 
system  by  penetration  and  proliferation  of  bacilli ;  since,  however,  the  clinical 
picture  of  this  disease  in  no  way  agrees  with  that  of  the  other  neuritides,  but 
presents  considerable  similarity  to  that  of  syringomyelia,  we  will  put  off  its 
consideration  to  the  chapter  devoted  to  this  disease  of  the  spinal  cord  (Lec- 
ture X). 

Cachexia  and  autointoxication  also  furnish  a  frequent  basis  for  the  devel- 
opment of  neuritic  phenomena:  carcinomatosis,  diabetes,  gout,  anemia,  senile 
marasmus,  pregnancy,*  leucemia,  chlorosis.  By  local  poisoning,  neuritis  occurs 
after  ether  injections  in  the  neighborhood  of  a  nerve,  by  local  infection  (with 
bacterial  invasion  which  often   ascends   along  the  nerves)    in  connection   with 

*  The  very  frequent  accompaniment  of  the  neuritides  of  pregnancy  by  pernicious  vomiting 
(hyperemesis  gravidarum),  speaks  for  their  autotoxic  origin. 


DISEASES    OF    THE    PERIPHERAL    NERVES  5 

septic  wounds,  phlegmons,  etc.,  by  local  disturbance  of  nutrition,  in  the  distribu- 
tion of  vessels  with  arteritic  or  arteriosclerosic  lesions. 

It  is  unexplained  whether  the  form  of  polyneuritis  known  as  Beri-beri  or 
Kakke,  endemic  in  southeast  Asia,  Japan  and  Brazil,  is  to  be  attributed 
to  a  poison  which  is  formed  outside  the  body  and  introduced  with  the  rice 
eaten,  or  to  a  toxine  formed  in  the  body  itself  by  microbes  which  have  entered 
it,  that  is,  whether  this  affection  is  to  be  counted  among  the  toxic  or  among 
the  infectious  neuritides. 

Finally,  after  traumatic  and  neuritic  influences,  the  third  and  last  category 
of  causes  of  disturbances  of  conduction  in  the  peripheral  nerves,  tumor  forma- 
tion, must  be  considered.  In  this,  mainly  true  neoplasms,  seldom  the  infectious 
granulomata — for  example,  gummata — are  concerned.  Carcinomata  frequently 
involve  the  neighboring  nerve  trunks,  cancer  metastases  in  the  axilla  for 
example  attacking  the  brachial  plexus.  Sarcomata,  fibromata,  myxomata, 
lipomata,  may'  arise  from  the  nerve  itself.  These  are  designated  as  "neuro- 
mata," though  it  is  not  entirely  proven  that  the  nerve  tissue  itself  takes  part  in 
the  tumor  formation.  However,  the  nerve  connective  tissue  plays  the  chief 
part  in  the  neoplastic  proliferation. 

Besides  solitary  "neuromata,"  *  there  are  plexiform  neuromata  which  de- 
velop along  the  whole  of  a  nerve  trunk  or  branch,  and  finally  a  "general  neuro- 
matosis" (Recklinghausen's  disease).  While  these  latter  often  cause  pain,  it 
is  remarkable  that  they  only  infrequently  cause  any  considerable  disturbance 
of  conduction  in  the  affected  nerves,  though  on  account  of  the  deformity  which 
they  produce  and  also  for  the  reason  that  fibromatous  growths  show  consid- 
erable tendency  to  undergo  sarcomatous  degeneration,  they  are  of  surgical 
importance. 

Pathogenesis    and  Pathological   Anatomy 

The  injury  of  a  nerve  at  any  part  of  its  course,  has  as  a  result  degenerative 
changes  in  its  parenchyma:  the  axis  cylinder  shows  the  earliest  changes,  it 
becomes  tortuous,  varicose,  and  at  length  breaks  up  into  very  fine  granules. 
Soon  after,  the  medullary  sheath  begins  to  undergo  changes,  the  myelin  of 
which  it  is  composed  breaks  up  into  fatty  droplets  which  are  at  length  absorbed. 
Certain  proliferative  changes  in  the  interstitial  tissues  proceed  parallel  with 
the  degeneration  of  the  parenchyma;  the  sheath  of  Schwann  shows  considerable 
increase  in  its  nuclei,  which  arrange  themselves  in  columns. 

According  to  the  law  of  Waller,  "A  nerve  filler  can  only  preserve  its 
anatomical  and  physiological  integrity,  while  it  remains  in  uninterrupted  con- 
nection  with  its  Living  cell  of  origin"-  the  degeneration  described  occurs 
throughout  the  peripheral  portion  of  (he  severed  nerve.  The  same  changes 
are  Usually    found,   also,    for  some  distance   in    the  central   stump. 

Of  course,  the  same  lesions  are  found  in  man  after  the  severing  of  a  nerve; 
simple    squeezing,    or    compression    which    lasts    longer,    produce    in    the    nerve 


ill    .  which  have  their  seat    upon  the  smallest  skin  nerves  seldom  give  any  other  symptoms 
than  mure  ur  Irs-,  sensitiveness  to  pressure;  these  .ire  the  so-called  "tubercula  dolorosa"  of  the 

surgeons. 


6  LECTURE    I 

changes  of  the  same  character,  but  quantitatively  less  severe.  However,  the 
anatomical  picture  produced  by  toxemic  injury,  refrigeration,  etc.,  is  often 
so  similar  to  the  traumatic  nerve  degeneration,  that  Babinski  could  remark, 
that  apart  from  tumor-formation,  all  diseases  of  the  peripheral  nerves  are  to 
be  attributed  to  neuritis.  In  spite  of  this,  I  cannot  decide,  like  other  neurolo- 
gists, to  denominate  the  disturbances  of  conduction  after  external  violence  as 
traumatic  neuritis,  and  on  this  account,  in  speaking  of  etiology,  I  kept  the  two 
categories  sharply  separated.  For  the  rest  there  are  acute  infections  and  toxic 
neuritides,  in  which  the  inflammatory  nature  of  the  process  is  quite  plainly 
marked  by  extensive  infiltration  of  the  connective  tissue  endoneurium,  epineu- 
rium  and  perineurium,  by  hyperemia  of  the  nerve,  diapedesis  of  white  corpuscles, 
etc.  Of  clinical  importance  is  the  tendency  of  the  sheath  of  Schwann  to 
undergo  proliferatory  processes  as  already  mentioned;  the  nuclei  arranging 
themselves  in  rows,  form  in  the  affected  parts  columns,  along  which  from  the 
central  stump  regeneration  of  the  axis  cylinder,  followed  by  that  of  the  medul- 
lary sheath,  can  occur.  In  severed  nerves,  regeneration  is  possible  even  years 
later,  as  is  shown  by  the  results  of  reunion  of  the  cut  fibers  by  nerve  suture 
undertaken  long  after  the  injury. 

The  General  Symptomatology  of  Interruption  of  Conduction 

Since  the  peripheral  nerves  have  to  conduct  stimuli  of  varied  kinds,  cen- 
tripetally  the  different  forms  of  sensation,  centrifugally  motor,  trophic  and 
vasomotor  impulses,  and  since  disturbance  of  their  capacity  to  conduct  can 
impair  any  of  these,  it  is  necessary  here  to  present  to  you  a  short  review  of  the 
clinical  physiology  of  each  of  these  functions. 

I.    SENSIBILITY 

This  term  in  no  way  expresses  a  simple  conception.  The  clinic,  however, 
does  not  go  so  far  in  its  analysis  as  experimental  physiology  which  differen- 
tiates a  sense  of  pressure,  sense  of  position,  sense  of  cold,  sense  of  heat,  sense 
of  pain,  sense  of  movement,  etc.  It  limits  itself  rather  in  the  testing  of  "general 
sensibility"  (i.e.,  the  afferent  impulses  which  do  not  come  from  the  eye,  from 
the  ear  and  from  the  gustatory  and  olfactory  apparatus)  to  the  investigation 
of  four  chief  qualities. 

a.  Touch  sense.  We  test  this  by  stroking  the  part  with  a  wisp  of  cotton, 
a  brush,  the  finger,  etc.  Its  diminution  is  called  tactile  lvypoesthesia,  its  loss, 
tactile  anesthesia.  In  pathological  increase  of  sensitiveness  to  touch,  whereby 
this  is  found  painful,  we  speak  of  tactile  hyperesthesia. 

b.  Temperature  sense.  To  study  this,  we  test  the  ability  of  the  subject  to 
distinguish  between  cold  and  warm  objects.  The  condition  of  impaired  or 
absent  temperature  sense  is  called  thermohypoestbesia  or  thermoanesthesia  as 
the  case  may  be.     The  opposite  condition  is  thermic  hyperesthesia. 

c.  Pain  sense.  Pinpricks,  pinching  of  a  fold  of  skin,  etc.,  furnish  infor- 
mation as  to  this.  Diminution  =  h'ypalgesia.  Loss  =  analgesia.  Increased 
sensibility  to  pain  is  called  hyperalgesia. 


DISEASES    OF    THE    PERIPHERAL    NERVES  7 

d.  Deep  sensibility  (also  "Bathyesthesia").  In  contradistinction  to  the 
previously  enumerated  "Superficial-sensibilities,"  whose  seat  is  in  the  integu- 
ment, we  understand  under  the  above  name  the  sum  of  the  centripetal  stimuli, 
which  stream  to  our  central  nervous  organs  from  the  muscles,  tendons,  bones, 
joints,  etc.  A  part  of  these  pass  the  threshold  of  consciousness  in  the  brain, 
and  inform  us  as  to  the  position  of  our  limbs,  the  angles  at  which  our  joints 
are  bent  (sense  of  position),  the  extent  of  a  movement  which  is  being  carried 
out  (sense  of  movement),  etc.  Here  belongs  also  vibration-sense  (palles- 
thesia), which  is  felt  when  a  vibrating  tuning-fork  is  placed  upon  a  super- 
ficially seated  part  of  the  skeleton.  Another  part  of  these  influences,  however, 
does  not  come  into  consciousness,  but  regulates,  subconsciously,  the  motor 
mechanisms,  which  come  into  action  in  all  complicated  and  combined  move- 
ments, such  as  walking  and  standing.  It  makes  possible  in  this  way  the  main- 
tenance of  equilibrium,  the  harmonious  course  of  locomotion,  the  synergistic 
action  of  related  muscle  groups,  etc. 

More  or  less  marked  defects  in  deep  sensibility  manifest  themselves  by  the 
symptoms  of  ataxia  (hypotaxia),  incoordination,  asynergy,  etc.  Since  in  rec- 
ognizing an  object  by  feeling  it  with  closed  eyes,  the  sense  of  position  and 
sense  of  movement  of  our  fingers  plays  the  chief  part,  stereoanesthesia  ( inabil- 
ity to  recognize  form  by  palpation)  is  also  an  expression  of  disturbed  deep 
sensibility.  Loss  of  vibration-sense'  is  called  pallanesthesia.  As  Head  has 
shown,  the  fibers  conveying  deep  sensibility  run  in  the  muscle  nerves,  on  which 
account  they  are  uninfluenced  in  lesions  of  the  cutaneous  nerve  branches. 

II.    MOTILITY 

Under  motility  in  the  broader  sense,  we  understand,  not  only  voluntary 
movements,   but.  also   the   phenomena   of  the   tonus   and   the   reflexes. 

a.  Voluntary  movement.  Complete  abolition  of  the  ability  to  perform 
voluntary  movements,  we  denominate  paralysis,  partial  disability,  paresis.  We 
Bpeak  of  a  paralysis  of  individual  muscles  when  a  few  muscles,  of  plegias  when 
whole  limbs,  or  at  least  portions  of  limbs,  are  affected;  if  a  single  extremity 
i-  involved  we  call  it  a  monoplegia,  if  two  extremities  on  the  same  side,  a 
hemiplegia,  if  two  symmetrica]  extremities,  a  diplegia  (when  it  is  the  two  legs 
the  term  paraplegia  is  used)  ;  an  extension  to  three  or  to  all  four  limbs  becomes 
respectively  triplegia  and  tetraplegia. 

h.  Tonus  and  Reflexes.  A  mechanical  stimulus  (striking  or  stroking)  ap- 
plied to  certain  parts  of  our  bodies  (certain  tendons,  bones,  skin  regions) 
under  normal  conditions  produces  certain  definite  motor  phenomena  (a  contrac- 
tion of  certain  muscles);  we  speak  of  them  as  reflexes.  Besides  Ibis,  there 
stream  (probably  from  tin;  whole  surface  of  our  bodies  and  from  all  the 
skeleton)  to  our  central  organs,  continued  subconscious  centripetal  stimuli  of 
little  intensity,  which  by  reflex  action  keep  up  a  moderate  continuous  contrac- 
tion of  all  our  muscles.  This  is  tin1  tonus.  We  can  define  this  in  the  follow- 
ing ti  mis:  "The  definite  degree  of  tension  which  gives  our  muscles  the  ability 
to  reply  promptly  with  a  contraction  to  voluntary  impulses  which  reach  them." 
That  in   normal   life,   the  stimuli   which   produce  the  tonus  are  continually   at 


8 


LECTURE    I 


work  is  proved  by  the  fact  that  in  healthy  persons  the  tonus  is  never  relaxed, 
even  in  sleep.  The  muscles  of  the  sleeper  are  never  so  completely  relaxed  as 
in  the  corpse;  and  only  in  an  advanced  stage  of  narcosis  does  the  tonus  give 
way.  Abolition  of  the  reflexes  and  of  the  tonus  we  call  respectively  areflexia 
and  atonia;  diminution  of  these,  hypo-reflexia  and  hypotonia;  for  the  exag- 
geration of  these  phenomena  on  the  other  hand  the  expressions  hyper-reflexia 
and  hyper-tonia  are  used. 

I  have  prepared  a  table  in  which  the  different  reflexes  and  the  methods  by 
which  they  are  elicited  are  exposed,  in  which  the  differentiation  between  skin, 
tendon,  and  bone  reflexes  is  shown,  and  the  most  important  reflexes  are  empha- 
sized. One  can  expect  to  elicit  all  the  reflexes  only  in  very  young  persons.  After 
puberty  the  great  majority  of  them  cannot  be  obtained  even  in  perfectly 
healthy  individuals.  As  to  the  condition  of  tonus,  we  estimate  it  partly  by 
determining  the  tension  and  resistance  of  the  muscles  by  palpation,  partly  by 
noting  the  resistance  which  is  opposed  to  passive  movements. 


Skin  Reflexes. 


Method  of  Eliciting. 


2.  Biceps  reflex 

3.  Triceps    reflex 

4.  Scapulo-hume- 

ral   reflex 

5.  Radius  reflex 

6 

7 

8 

9.1 

10-3  

11 

12.  Patellar  reflex 

13 

14.  Achilles  reflex 

15 

16 


Scapular  reflex 


Palmar  reflex 
Epigastric  reflex 

Upper  abdominal   reflex 

.Middle  abdominal  and 
lower  abdominal  re- 
flex 

Cremasteric  reflex 


Gluteal  reflex 


Plantar  reflex 
Anal  reflex 


Irritating  the  skin  over 
the  scapula 

Striking  upon  the  biceps 
and  tendon 

Striking  upon  the  tri- 
ceps  tendon 

Striking  upon  the  infe- 
rior internal  angle  of 
the  scapula 

Striking  upon  the  sty- 
loid process  of  the 
radius 

Irritation    of   the    palm 

Stroking  from  the  mam- 
ma downward 

Stroking  the  skin  of  the 
upper  abdomen 

Stroking  the  skin  of  the 
abdomen  in  the  mid- 
dle and  lower  parts 

Stroking  the  inner  side 
of  the  thigh. 

Striking  upon  the  quad- 
riceps tendon 

Stroking  the  nates 

Striking  upon  the  Achil- 
les tendon 
Stroking  the  sole 
Irritating  the  perineum 


Contraction  of  the  mus- 
cles over  the  scapula 
Flexion   of   the    forearm 

Extension    of    the    fore- 


Supination  of  the  fore- 
arm 

Flexion  of  the  fingers 

Retraction  of  the  epi- 
gastrium 

Retraction  of  the  ab- 
domen 

Retraction  of  the  ab- 
domen 

The    testicle     is     jerked 

upward 
Extension  of  the  leg 

Contraction   of   the   glu- 
teal muscles 
Extension  of  the  foot 

Flexion  of  the   toes 
Contraction    of    the    ex- 
ternal    sphincter     ani 
muscle 


III.     TROPHIC    FUNCTIONS 

The  Law  of  Waller  already  mentioned  applies  also  to  the  muscles,  for 
whose  anatomical  integrity  an  uninterrupted  connection  with  the  cells  of  the 
anterior  horns  of  the  spinal  cord,  or  in  the  case  of  those  innervated  by  the 
cranial  nerves,  with  the  motor  nuclei  of  the  brain  axis,  through  their  nerves 


DISEASES    OF    THE    PERIPHERAL    NERVES  9 

of  supply,  is  presupposed.  If,  in  a  motor  nerve,  conduction  is  broken,  the 
muscles  supplied  by  it  undergo  a  degenerative  process  which  we  call  neurogenic 
degenerative  atrophy.  Histologically  this  is  characterized  by  the  disappearance 
of  the  contractile  element  (by  granular,  albuminous  and  fatty,  less  frequently 
by  hyaline  and  vacuolar,  degeneration),  and  its  replacement  by  fatty  and 
connective  tissue.  These  degenerative  processes,  under  conditions  which  admit 
of  a  reestablishment  of  the  connection  between  the  muscles  and  the  tropho- 
motor  cells  of  the  central  nervous  system,  can  be  repaired  by  regenerative 
processes ;  the  histological  criteria  of  these  last  ai-e,  among  other  things,  the 
proliferation  of  the  nuclei  of  the  sarcolemma  surrounding  the  muscle  fibers, 
the  appearance  of  polynuclear  giant  cells,  "myoblasts,"  from  which  muscle 
fibrils  are  formed  anew,  the  presence  of  very  voluminous  muscle  fibers,  etc.  A 
clinical  sign  of  neurogenic  atrophy  is  the  notable  electro-physiological  altera- 
tion, first  carefully  studied  by  Wilhelm  Erb  in  1872,  and  called  by  him  "Reac- 
tion of  Degeneration"  (R.  D.).  To  the  symptomatology  of  this  we  will  return 
farther  on  in  this  lecture.  Besides  this,  the  central  nervous  system  through 
the  peripheral  nerves  exerts  a  trophic  influence  upon  the  skeleton ;  when  this 
is  cut  off  in  growing  individuals,  there  is  more  or  less  limitation  of  further  bone 
growth  in  the  affected  territory.  Abnormal  fragility  of  bones  can  also  occur 
under  these  conditions. 

IV.     VASOMOTOR   FUNCTIONS 

Experimental  physiology  teaches  us  that  two  sorts  of  fibers  pass  from  the 
sympathetic,  through  the  peripheral  nerves  to  the  blood  vessels,  those  which 
narrow  ("vasoconstrictors")  and  those  which  increase  ("vasodilators")  the 
calibre  of  the  vessels. 

Dilator  fibers  indeed  are  only  recognizable  in  a  few  nerves,  for  example,  in 
the  sciatic.  Vasoconstrictor  fibers  on  the  contrary  are  quite  generally  recog- 
nized as  constituents  of  peripheral  nerves.  Nevertheless,  in  man,  only  rarely 
(that  is  only  in  the  earliest  stages)  are  we  able  to  observe  the  picture  which  the 
physiologist  sees  in  animals  after  destruction  of  the  vasoconstrictor  elements, 
namely,  redness  and  heat  of  the  skin.  Much  more  common  is  the  apparently 
opposite  picture  which  can  follow  such  an  injur}',  or  even  without  one  being 
definitely  determined.  Namely,  the  integument  of  the  parts  cut  off  from 
vasomotor  innervation  becomes  cyanotic  and  cold.  The  cyanosis  is  explained 
by  the  chronic  inhibition  of  the  capillary  circulation,  on  account  of  the  removal 
of  the  vascular  tonus,  the  coldness,  by  the  changes  in  nutrition  of  the  skin 
resulting  from  this  cyanosis.  The  mechanism  of  the  sweat  secretion  is  very 
similar  to  that  of  the  vasomotor  innervation,  the  secretory  fibers  being  mostly 
closely  mixed  in  with  the  vasomotor  fibers.  Their  destruction  has  as  a  result, 
lessening  or  inhibition  of  tin-  sweat  secretion  (hypoidrosis  or  anidrosis).  The 
opposite  anomaly  is  called  hyper-idrosis. 

After  this  general  diagnostic  excursion,  we  will  proceed  to  the  description 
of  the  sensory  symptoms  which  accompany  the  disturbances  of  conduction 
in  peripheral   nerves. 

Apart  from  traumatic  severing  of  nerves,  there  is  seldom  total  anesthesia 


10  LECTURE    I 

of  the  integument,  more  usually  there  is  a  general  reduction  of  superficial 
sensibility.  Very  frequently,  however,  the  different  qualities  of  sensation  are 
affected  in  different  degrees,  so,  for  example,  touch  sense  is  intact,  while  pain 
and  temperature  senses  are  diminished.  In  neuritides,  very  often  the  dis- 
turbances of  sensibility  are  not  equally  distributed  over  the  whole  area  supplied 
by  the  affected  nerves,  but  increase  in  intensity  toward  the  periphery,  which 
is  perhaps  connected  with  the  increased  distance  of  the  parts  there  situated 
from  the  trophic  centers.  In  diseases  of  a  number  of  nerves,  as  in  neuritis  of 
plexuses  and  in  polyneuritis,  bathyanesthesia  or  bathyhypoesthesia  can  make 
itself  felt  in  disturbances  of  sense  of  position  or  of  movement,  namely  as  ataxia 
or  incoordination.  A  disturbance  of  vibration-sense  is  recognizable  when  cer- 
tain nerves  are  put  out  of  function,  particularly  over  the  corresponding  bones. 
Disturbances  of  deep  sensibility  are  usually  accompanied  by  reduction  or  loss 
of  tendon  reflexes. 

The  motor  symptoms  in  destructive  lesions  of  nerves,  consist  in  losses 
of  function,  varying  from  slight  paresis  to  complete  paralysis.  They  are 
always  accompanied  by  reduction  of  muscular  tonus  with  regional  hyporeflexia 
or  areflexia,  with  more  or  less  marked  neurogenic  muscular  atrophy  and  with 
the  reaction  of  degeneration  already  mentioned,  which  will  now  be  discussed. 

THE    REACTION    OF    DEGENERATION    (Erh,    1872) 

Let  us  first  get  clearly  the  normal  electric  reactions  of  the  human  muscles 
as  they  are  brought  out  in  the  usual  clinical-neurological  examination.  In 
this,  we  use  the  so-called  unipolar  method  of  stimulation,  in  which  a  large 
("'indifferent")  electrode  is  placed  upon  the  chest  or  back  of  the  person  being 
examined,  a  smaller  one  (as  a  rule  the  3  cm  square  electrode,  Stmt  zing's 
"Normal  electrode")  upon  the  muscles  or  nerves  to  be  tested.  Contraction  of 
muscles  occurs  with  the  Galvanic  current,  not  when  it  is  slowly  raised,  but  on 
sudden  variations  of  current  strength  (that  is,  on  making  or  breaking  the 
galvanic  circuit,  naturally  also,  upon  conducting  through  them  the  faradic 
current  with  its  rapid  alternate  making  and  breaking).  With  a  weak  galvanic 
current,  a  contraction  is  obtained  only  when  the  negative  pole  is  used  as 
stimulating  electrode,  and  the  current  is  closed ;  Kathodal  closing  contraction 
(KCC).  If,  now,  the  current  is  made  stronger  and  stronger,  we  obtain  next 
an  Anodal  closing  contraction  and  an  Anodal  opening  contraction  (AnCC  and 
AnOC),  (the  sequence  of  these  two  reactions  is  different  in  different  individ- 
uals, AnCC  usually  appears  before  AnOC),  only  with  much  stronger  current 
is  a  Kathodal  opening  contraction  (KOC)  obtained.  If,  now,  with  the  current 
strength  which  has  sufficed  to  produce  KOC,  the  current  is  closed,  the  negative 
pole  being  on  the  point  to  be  stimulated,  we  get,  not  a  short  contraction,  but 
one  which  persists  while  the  electrode  is  applied,  Kathodal  closing  tetanus 
(KCTe).  A  still  more  powerful  current  is  needed  to  produce  Anodal  closing 
tetanus  (AnCTe),  while  in  normal  man,  Anodal  opening  tetanus  (AnOTe) 
does   not   occur. 

It  is  self-evident  that  KOC  can  only  occur  when  the  muscle  was  not  just 
before  the  interruption  of  the  current  in  a  condition  of  tetany;  KCTe  can  be 


DISEASES    OF    THE    PERIPHERAL    NERVES 


n 


avoided  even  with  a  very  strong  current,  however,  if  with  careful  avoidance  of 
sudden  current  variations,  the  number  of  milleamperes  necessary  to  produce 
KOC  are  gradually  introduced  by  use  of  the  rheostat.  Finally  it  must  be 
added  that  between  the  results  of  '"indirect"  (through  the  nerve)  and  "direct" 
(to  the  muscle  itself),  galvanic  stimulation,  there  is  this  difference,  that  in 
the  last  method,  the  effect  of  opening  the  circuit  is  generally  less. 

In  "Reaction  of  degeneration"  (the  clinical  physiological  criterion  of  dis- 
turbed or  interrupted  trophic  connection  between  nervous  system  and  muscle), 
we  find  in  contradistinction  to  the  normal  relations  described  above,  the  fol- 
lowing phenomena.  The  galvanic  and  faradic  irritability  through  the  nerve 
grow  less  and  less,  until,  in  case  there  is  complete  interruption  of  conduction 
in  the  nerve  supplying  the  affected  muscle,  by  the  end  of  fourteen  days  it  has 
completely   disappeared.      Direct   faradic   irritability  of  the  muscle  acts   sim- 


10, 

20. 

30. 

W.            50-            60.           70.           80. 

week. 

W. 

W- 

w.          w.         w«         w.         w. 

,'' 

-**„ 

*,^ 

___  Direct  galvanic  irritability. 

0 
t 

*^ 

**^ 

mum  Direct  faradic  irritability. 

* 

H, 

** 

\ 

Indirect  galvanic  and  faradic 
irritability. 

*x 

\ 

^ 

s 

x 

x 

X. 

s 

*x^ 

i! 

xx. 

'k^ 

Electric  Irritability  in  "Reaction  of  Degeneration.' 


ilarly.  Direct  galvanic  irritability,  on  the  contrary,  first  increases,  to  fall 
again  at  the  end  of  about  two  months,  in  case  the  trophic  connection  is  not 
in  tlie  meantime  reestablished.  If  conduction  is  later  renewed,  galvanic  irrita- 
bility and  later  faradic  irritability  of  the  muscle  direct,  come  back,  and  at 
length  electric  stimulation  of  the  nerve  is  again  followed  by  contraction.  If 
conduction  is  not  restored,  however,  after  from  one  to  one  and  a  half  years, 
even  galvanic  irritability  of  the  muscle  is  permanently  lost. 

With  these  quantitative  anomalies  of  irritability,  qualitative  changes  go 
hand  in  hand.  There  is  an  alteration  of  Hie  character  of  the  contraction 
on  galvanic  stimulation;  instead  of  the  normal  quick  contraction  we  get  a 
slow  "vermicular"  response,  further  a  modification  of  the  so-called  contraction 
formula.  While  normally  KCC  appe.-us  with  a  weaker  current  than  AnCC 
in  reaction  of  degeneration  this  relationship  is  reversed.  We  indicate  this 
by  the  formula  AnCC>KCC.  Instead  of  complete  reaction  <>t'  degeneration, 
we  find  in  slight  neurogenic-trophic  disturbances  of  the  muscles  different 
Varieties   of  "partial    R.    D.,"   to   consider   all    of  which    would    fake   us    too    I'm 


12 


LECTURE    I 


afield.  Stintzing  has  distinguished  no  less  than  thirteen  varieties  of  partial 
R.  D.  We  must  content  ourselves  with  a  description  of  the  most  frequent 
of  these  varieties.  In  this  nerve  irritability  is  preserved,  usually  faradic  irrita- 
bility of  the  muscle  also,  but  there  is  galvanic  overirritabilty,  reversal  of  the 
polar  formula  and  as  the  most  important  criterion  of  the  R.  D.,  slow  character 
of  the  contraction.  While  in  complete  R.  D.  slow  return  to  the  normal — in 
case  this  occurs — requires  from  three  to  seven  months,  in  partial  R.  D. 
under  proper  treatment  restitution  can  begin  in  six  weeks.  Very  rapid  recov- 
ery can  only  be  expected  in  such  peripheral  paralyses   in  which  R.  D.   does 


KAKAKAKAKAKA 


Fig.  2. 

Myographic  Curves  on  Direct  Stimulation  of  a  Muscle  with  the  Galvanic  Current. 

K  =  Cathode;  A  =  Anode.     Upper  curve,  normal  reaction;  lower  curve,  "Reaction  of 

Degeneration." 


not  appear,  however.     The  normal  irritability  of  the  most   important  nerves 
and  muscles  is  shown  in  the  following  tables. 

According  to   Stintzinc/,   the   nerves   can   be   arranged   according  to   their 
galvanic  irritability  in  milleamperes  as  follows: 


; 

Lower    Limit. 

Upper   Limit. 

Average. 

1. 

N. 

musculo-cuta- 

1. 

N. 

musculo-cuta- 

1. 

X. 

musculo-cuta- 

0.05 

i 

0.28 

neous   

0.17 

2_ 

N. 

accessorius. . 

0.10 

2 

X. 

accessorius  . . 

0.44 

2. 

X. 

accessorius  .  . 

0.27 

3. 

N. 

ulnaris  upper 

0.2 

3. 

X. 

ulnaris  upper 

0.9 

3. 

X. 

ulnaris  upper 

0.55 

4. 

N. 

0.2 

4. 

R. 

mentalis   .... 

1.4 

4. 

X. 

medianus. .  . . 

0.9 

5. 

N. 

medianus.  . .  . 

0.3 

5. 

X. 

medianus    . . . 

1.5 

5. 

R. 

mentalis 

0.95 

6. 

N. 

cruralis 

0.4 

6. 

X. 

1.7 

0. 

X. 

1.05 

7. 

N. 

0.4 

7. 

X. 

peroneus  .... 

2.0 

7. 

X. 

peroneus    . . . 

1.1 

8. 

R. 

mentalis 

0.5 

8. 

R. 

zvgomaticus  . 

2.0 

8. 

R. 

zygoniaticus  . 

1.4 

9. 

N. 

ulnaris  lower  0.6 

9. 

R. 

frontalis 

2.0 

9. 

R. 

frontalis   .... 

1.45 

10. 

R. 

zvgomaticus  . 

0.8 

10. 

X. 

tibialis    

2.5 

10. 

X. 

tibialis    

1.45 

11. 

R. 

frontalis 

0.9 

11. 

X. 

facialis   

2.5 

11. 

X. 

ulnaris    lower 

1.6 

12. 

X. 

radialis 

0.0 

12. 

X. 

ulnaris   lower 

2.6 

12. 

N. 

facialis 

1.76 

13. 

N. 

facialis 

1.0 

13. 

X. 

2.7 

13. 

N. 

1.8 

DISEASES    OF    THE    PERIPHERAL    NERVES  13 


TABLE  SHOWING  THE  IRRITABILITY  OF  THE  MUSCLES  ACCORDING  TO 
STINTZING. 

Galvanic  Irrita-        Size  of  the  Elec- 
Muscle.  bility  in  M.  A.  trode  in  cm'- 

M.  trapezius    1.6  1- 

M.  deltoides    1 5-2.0  12 

M.  pectoralis  major   0.4  6 

M.  pectoralis  minor  0.1—2.5  6 

M.  serratus  magnus   1.0-8.5  12 

M.  brachialis  anticus   1.1-1.7  3 

M.  extensor  communis  digitorum  0.6-3.0  3 

M.  extensor  carpi  radialis   0.8  3 

M.  extensor  brevis  pollicis   1.5-3.5  3 

M.  pronator  radii  teres  2.5  S.8  3 

M.  flexor  sublimis  digitorum   0.3-1.5  3 

M.  flexor  carpi  ulnaris   0.9-2.9  3 

M.  abductor  minimi  digiti  2.5  3 

M.  rectus  femoris   1.6-6.0  20 

M.  vastus  internus   0.3-1.3  20 

M.  tibialis   anticus    1.8-5.0  1 2 

Finally  as  concerns  the  trophic  and  vasomotor  disturbances,  in  destructive 
diseases  of  the  peripheral  nervous  system,  they  are  less  regularly  found  than 
might  be  expected  from  the  fact  that  through  the  "gray  rami  communicantes," 
sympathetic  fibers  run  into  all  the  peripheral  nerves.  As  already  said,  redness 
and  local  raise  of  temperature  occur  only  as  fleeting  symptoms,  later  in  the 
place  of  hyperemia,  there  is  cyanosis  and  abnormal  coolness  of  the  integument. 
Local  hyperidrosis  is  not  infrequent.  I  will  mention  now  the  oedema  which 
occurs  in  infectious  polyneuritis.  In  beri-beri  this  symptom  is  so  frequent, 
that  we  can  speak  of  a  hydropic  form  of  this  tropical  disease,  the  "moist"  form 
of  beri-beri.  The  skin  in  all  forms  of  breach  of  conduction  can  be  shiny  and 
atrophic  (the  "glossy  skin"  of  the  English),  and  shows  then  an  increased 
vulnerability,  so  that  the  smallest  injuries  will  not  heal.  Further,  vesicular 
eruptions  in  the  skin  areas  deprived  of  innervation,  also  falling  off  of  nails, 
collections  of  air  in  the  nail  substance,  hypertrichosis  or  falling  out  of  the  hair, 
and  retardation  of  growth  or  thinning  of  bony  parts  are  sometimes  seen. 

SPECIAL   SYMPTOM ATOLOCxY  OF  THE   DEFECTS   OF  FUNCTION 
IX  Till:   DIFFERENT  PERIPHERAL   NERVES 

In  order  to  understand  the  motor  and  sensory  symptoms  corresponding  to 
lesions  in  the  territories  of  distribution  of  definite  nerves,  an  accurate  acquaint- 
ance with  the  functions  of  the  various  peripheral  nerves,  as  well  as  with  those 
of  the  muscles  supplied  by  them  is,  of  course,  necessary. 

A  synoptic  table  which  will  present  this  information  most  expeditiously 
and  in  most  condensed  form  is  hence  introduced  at  this  point. 


14 


LECTURE    I 


A.  Plexus  eervicalis 

Nervi  cervicales 
N.  phrenicus 


I.     The  Spinas  Nerves 
1.     Motor  Functions 


Musculi  profundi  colli  Flexion,  extension,  turning  the  neck 

Mm.  scaleni  Raising  the  ribs  on  inspiration 

Diaphragm  Inspiration 


B.  Plexus  brachialis 

N.  thoracicus   ant. 


M.  pect.  maj.  and  min. 


N. 

thoracic,  long. 

M.  serratus    magnus 

N. 

dorsalis  scap. 

M.  levator  scapulae 
Mm.  rhomboidei 

N. 

suprascap. 

M.  supraspinatus 
M.  infraspinatus 

N. 

subscapular 

M.  latissimus   dorsi 
M.  teres  major 
M.  subscapularis 

N. 

circumflex  (axillaris) 

CM.  deltoid 

(  M.  teres  minor 

N. 

musculo-cutan. 

M.  biceps  bracchi 
M.  coraco-brachialis 
M.  brachialis  anticus 

N. 

medianus 

M.  flexor  carpi   radial 

N.  medianus 


N.  ulnaris 


Nervus   radialis    (muscu- 
lo-spiral) 


M.  palmaris  longus 

M.  flexor   sublim.   digitor 

M.  flexor  long,  pollicis 
M.  flexor  prof,  digit  (ra- 
dial half) 
M.  pronator  radii  teres 
M.  abductor  brev.  pollicis 
M.  flex,  pollicis  brev. 
M.  opponens  pollicis 
M.  flexor  carpi   ulnaris 

M.  flexor  digit,  prof,  (ul- 
nar half) 
M.  adductor  pollicis 
Mm.  hypothenares 

Mm.  lumbricales 

Mm.  interossei 

M.  triceps  bracchi 

M.  supinator  longus* 


Adduction,  and  drawing  the  arm  down- 
ward and  forward 

Fixation  of  the  scapula  on  raising  the  arm 

Raising  the  scapula 

Drawing  the  scapula  upward  and  inward 

Elevation  and  outward  rotation  of  the 
arm 

Outward  rotation  of  arm 

Inward  rotation  and  adduction  of  the 
arm  toward  the  back 

Inward   rotation  of  arm 

Raising  the  arm  to  a  horizontal  position 

Outward  rotation  of  the  arm 

Flexion  and  supination  of  the  forearm 

Elevation  and   adduction  of  the  forearm 

Flexion  of  the  forearm 

Flexion  and  drawing  to  the  radial  side 
of  the  hand 

Flexion  of  the  hand 

Flexion  of  the  middle  phalanges  of  fin- 
gers II-V 

Flexion  of  the  end  phalanx  of  the  thumb 

Flexion  of  the  end  phalanges  of  fingers 
II   and  III 

Pronation  of  hand 

Abduction  of  metacarp.  I 

Flexion  of  proximal  phalanx  of  thumb 

Opposition  of  metacarp.  I 

Flexion  and  drawing  to  the  ulnar  side 
of  the  hand 

Flexion  of  end  phalanges  of  fingers  IV 
and  V. 

Adduction  of  metacarp.  I 

Abduction,  opposition,  flexion  of  little 
finger 

Flexion  of  proximal,  extension  of  other 
phalanges 

Same  action  as  preceding,  also  spreading 
apart   and   bringing  together   fingers 

Extension  of   forearm 

Flexion  of  forearm 


*  The  term  "supinator  longus"  is,  in  fact,  incorrect,  since,  as  shown  by  electric  stimulation, 
the  muscle  has  no  effect  in  causing  supination,  but  produces  rather  slight  pronation.  The  des- 
ignation "brachloradialis"  would  hence  be  preferable,  but  though  used  by  anatomists,  it  has 
never  been  adopted  by  clinicians. 


DISEASES    OF    THE    PERIPHERAL    NERVES 


15 


B.  Plexus  brachialis 


C.  Nervi  thoracales 

D.  Plexus  lumbalis 
N.  cruralis 

N.  obturatorius 

E.  Plexus  sacralis 
X.  gluteus  sup, 

X.  gluteus  inf. 

X.  ischiadieus  (sciatic) 


(a)    X.   peroneus 
(«)   prof. 


(li)    superf. 
(b)    N.  tibialis   (ant.) 


M.  extensor  carpi  rad.         Extension  and  drawing  to  radial  side  of 

hand 
M.  extensor  digit,  comm.    Extension  of  proximal   phalanges   of  fin- 
gers II-V 
M.  extensor  minim,  digit.    Extension   of   proximal   phalanx   of   little 

finger 
M.  extens.  carp,  ulnar  Extension   and   drawing  to   ulnar  side  of 

hand 
M.  supinator  brevis  Supination   of  forearm 

M.  abductor  pollicis  long.    Abduction  of  metacarp.  I 
M.  extensor  pollicis  brev.    Extension  of  proximal  phalanx  of  thumb 
M.  extensor  pollicis  long.    Abduction  of  metacarp.  I   and  extension 

of  end  phalanx  of  thumb 
M.  extensor  indicis  prop.    Extension  of  proximal  phalanx  of  index 
finger 

Mm.   thoracis   et   abdom-    Raising   the    ribs,   expiration,    abdominal 
inis  pressure,  etc. 


M.  ileo-psoas 

M.  sartorius 

M.  quadriceps 

M.  pectineus 

M.  adductor  longus 

M.  adductor  brevis 

M.  adductor  magnus 

M.  gracilis 

M.  obturator  extern. 


M.  gluteus  med. 

\1.  gluteus  min. 

M.  tensor  fascia?  lata? 

M.  piriformis 

M.  gluteus  max. 
i~     M.  obturator  intern. 

Mm.   gemelli 
(     M.  quadratus   femoris 

M.  biceps  femoris 

.M.  semitendinosus 

M.  semimembranosus 

M.  tibialis  anticus 
M.  extens.   lung,  digit 

M.  extens.  halluc.  long. 

M.  extens.  digit,  brev, 

M.  extens.   halluc.  brev. 

Mm.  peronei 
M  gastrocnemius 
M.  soleus 

M.   tiliialis'posticus 
M.  flexor  long,  digit. 
M.  flex,  halluc.  long. 

M.   flex,  brev.  digit. 
M.   Ilex,   halluc.   brev. 

Mm.  plantares  pedis 


X.  pudendus 


Mm.    perim 
teres 


ri    gpbinc 


Flexion  of  the  thigh 
Inward  rotation  of  the  thigh 
Extension  of  leg  on  thigh 

Adduction  of  the  thigh 


Adduction    and    external   rotation   of   the 
thigh 


Abduction    and    inward    rotation    of    the 

thigh 
Flexion   of  the  thigh 
External  rotation  of  the  thigh 
Extension  of  the  thigh 

External  rotation  of  the  thigh 


Flexion  of  the  leg 

Dorsal  flexion  and  supination  of  the  foot 

Extension  of  the  toes 

Extension  of  great  toe 

Extension  of  tin-  toes 

Extension  'if  great  toe 

Dorsal  flexion  and  pronation  of  foot 

Plantar  flexion  of  the  foot 

Adduction  of  the  foot 

Flexion  of  the  end  phalanges  II-V 

Flexion    of  mil    phalanx    I 

Flexi f  tin-  middle  phalanges  II-V 

Flexion  of  middle  phalanx  I 

Spreading,   bringing   together   and    flexion 
of    Hie    proximal    phalanges    of    II"'    Iocs 

Sphincter  muscles  of  tin-  pelvic  organs, 
co-operation  in  the  sexual  act 


16 


LECTURE    I 


In  the  first  column  of  this  table  will  be  found  the  names  of  the  different 
nerves,  in  the  second,  those  of  the  muscles  supplied  by  them,  in  the  third  the 
movements  which  are  produced  when  these  muscles  act.  The  ability  or  in- 
ability to  carry  out  these  movements  informs  us  as  to  the  state  of  the 
respective  muscles,  whether  they  are  paralyzed  or  not.  The  investigation 
of  course  must  include  a  study  of  the  electric  contractility  as  well  as  that 
of  voluntary  movement.  For  this,  an  acquaintance  with  the  electric  motor 
points  of  the  muscles  is  necessary.  These  are  shown — as  they  usually  occur 
in  normal  persons — in  Figures  3  to  8.      In  the  investigation  of  pathological 


M.  splenius 
M.  sternocleidomastoideus 


M    levator  anguli  scapul 


N.  dorsalis  scapul 
N.  axill 


N".  thoracicus  longu; 
<M.  serratus  antictis  major} 


Plexus  briulnali: 


M.  masseter. 
N.  hypogloseus. 

M.  platysma  royoides. 
M.  sternohyoideus. 
M.  omohyoideus. 
M.  Bternothyreoideus. 


Erb's  Supraclavicular  point  CM.  deltoideus, 
biceps,  brachialis  internus  u.  supinator 
longus). 


Fig.  3. 
Motor  Points  of  the  Neck  and  of  the  Trigeminus.     Erb's  Supraclavicular   Point. 


cases  the  fact,  first  discovered  by  Wertheim-Salomonson,  that  in  muscles  show- 
ing RD.  there  is  "transposition  of  the  motor  points"  should  not  be  forgotten. 
The  affected  muscle  reacts  from  points  other  than  those  given  in  the  tables, 
usually  more  distally  located. 

2.      The  Most  Important  Types  of  Paralysis 

We  will  now  pass  briefly  in  review  the  clinical  pictures  of  the  most  important 
paralyses  of  the  spinal  nerves. 

a.  In  total  paralysis  of  the  brachial  plexus,  the  whole  arm  hangs  flaccid 
and  immovable,  the  muscles  of  the  shoulder  with  the  exception  of  the  trapezius, 


DISEASES    OF    THE    PERIPHERAL    NERVES 


17 


are  also  incapable  of  functionating.  In  the  upper  arm  type  (also  called  the 
Ducherme-Erb  type)  of  paralysis,  as  a  rule  only  the  biceps,  deltoid,  brachialis 
anticus  and  supinator  longus  are  affected:  elevation  of  the  arm  and  flexion  at 
the  elbow  are  hence  impossible.  Only  exceptionally  does  the  paralysis  affect 
one  or  more  of  the  following  muscles:  Infraspinatus,  latissimus  dorsi,  teres 
major,  pectoralis  major,  in  which  event  the  arm  hangs  down  in  a  position 
of  adduction  and  rotation  inwards.  The  lower  arm  type  of  paralysis 
(Klumphe's  paralysis)  is  rare.  It  is  often  limited  to  the  muscles  of  the  ball 
of  the  thumb  and  little  finger   and  to   the  interossei;  occasionally,   however, 


Oberster  Facial 
Facialiss' 
Mm. 
setattollensauricul 
M.  occipital 
Mittlerer  Facialisa 
M.  stylohyoid 
M.  dtgast 


M.  corrugator  supercilii. 
M.  orbicularis  palpebrarum. 

M.  compressor  nasi  etpyram. 
M.  levator  labii  sup-  alaequtj 
M.  levator  labii  6up.  proprii 
M    zygomaticus  minor. 
M.  dilatator   narium. 
JVI.  zygomaticu6  major. 


M.  levator  menti. 
M.  quadratus  metiti. 
M.  triangularis  mentl. 


Fig.  4. 
Motor  Points  of  the  Face. 


affects  some  of  the  flexor  muscles  of  the  forearm  and  on  account  of  accompany- 
ing lesion  of  the  sympathetic  fibers  (the  ramus  communicans  of  the  first  dorsal 
nerve)  is  apt  to  be  accompanied  by  a  narrowing  of  the  pupil  on  the  affected 
miIi     (paralysis   of  the  dilator   pupill.r  muscle). 

b.  In  paralysis  of  the  long  thoracic  nerve,  the  arm  can  no  longer  be  raised 
above  the  horizontal  by  the  'action  of  the  serratus  magnus  muscle:  if  the 
patient  extends  it  forward,  a  wing-like  deviation  of  the  scapula  from  the 
thorax  is  noticed  ("scapula  alata"  or  winged  scapula,  see  Fig.  9). 

c.  Paralysis  of  the  suprascapular  nerve,  on  account  of  the  loss  of  function 
01  the  supraspinatus  and  infraspinatus,  hinders  external  rotation  of  the  arm, 
which  can  be  very  disturbing,  for  instance  in  writing';  besides  this,  the  atrophy 
of  the  affected  muscles  is  very  apparent.     (See  Fig,  10.) 


18 


LECTURE    I 


N.  radialis.        M.  triceps  (cap.  ext.) 
M.  triceps 
M.  brachials  intern.    /  __(cap.  long.) 


M.  radial,  ext.  brev. 
M.  extens.  dig, 
M.  extens.  dig, 
M,  extens.  ind 
M.  abductor  pollicia  long. 


.  pollicia  brev; 
M.  extensor  pollicis  long. 


M.  abduct,  digit,  min.  (N.  ulnaris), 


Fig.  5. 
Motor   Points   of  the   Upper   Extremity,  Outer   Side. 


M.   deltoideus    (anterior  half),  N.   axillar 

N.  niusculo-cutaneus. 

M .  biceps  brachii. 

M.  brachial,  int. 


M.  pronator  teres.  M.  abductor  pollic.  brer. 

M.  flex.  dig.  comm.  prof.  |     M.  opponens  pollic 


M.  flex,  pollic.  brev. 

M.  adduct.  pollic.  brev, 
Mm.lumbr.LetH. 


Mm.  lumbr.  IlletrV. 
M.  opponens  dig. ; 
M.  flexor  dig.  min. 
M.  abduct,  dig.  nun. 
M.  pal  mam  brev. 


H.ul 


M.  flexor  carpi  ulnaris.  N.  ulnaris. 

Fig.  6. 
Motor  Points  of  the   Upper  Extremity,  Inner  Side. 


DISEASES    OF    THE    PERIPHERAL    NERVES 


19 


d.  Paralysis  of  the  circumflex  nerve  puts  the  deltoid  out  of  function ;  the 
arm  cannot  be  raised  either  laterally,  anteriorly  or  posteriorly.  The  rounding 
of  the  shoulder  disappears  and  the  contours  of  the  head  of  the  humerus  and 
the  acromion  process  become  visible.     ■ 

e.  Paralysis  of  the  musculocutaneous  nerve  causes  great  reduction  of  the 
ability   to  flex  the  forearm  at  the  elbow.     Fluxion   is  only  possible  with  the 


N.  obtu 

M.  pectineus 

M.  adductor  magnus 
AI.  adductor  longus 


M.  extens  digit,  conun.  brev 


N.  peron 
M.  tibialis  ant 


M.  peroneus  longus. 

M    peroneus  brevis 

tensor  hallucis  long 


M.  flexor  hallucis  long. 
M.  nhductor  digiti  mio. 


Mm.  interossei  dorsales. 


Fio.  T. 
Motor  Points  of  the  Lower  Extremity,  Anterior  View. 


forearm  in  pronation,  in  which  position,  I  lie  supinator  longus  and  the  flexors  of 
the  fingers  can  to  a  certain  extent   take   the  place  of  the  paralyzed   muscles 

(  biceps  and  liracliialis  anticus). 

/.      Paralysis   of   Hie   median    nerve   is   made  evident    by    I  lie    following   symp- 
toms:    Weakness    in    flexion    at     the    wrist    with    deflection    toward    the    ulnar   side; 

inability  to  bend  the  thumh  or  in  exert   its  action  as  opponent  of  the  fingers; 
substitution    of    inward    rotation    of    tin-    arm     for    pronation    of    the    forearm; 

interference  with  flexion  of  the  index  finger  (in  that   this  can  only  be  accom- 
plished in  the  metacarpophalangeal   joint  by  the  action  of  the  interosseus) ; 


20 


LECTURE    I 


considerable  impairment  of  the  power  of  grasping  and  holding  on  to  objects, 
for  which  only  the  three  fingers  on  the  ulnar  side  can  be  used. 

g.  Particularly  typical,  and  to  be  diagnosed  at  the  first  glance  is  ulnar 
paralysis.  (See  Figs.  11  to  13.)  Flexion,  especially  of  the  ulnar  side  of 
the  hand,  is  weak,  on  account  of  the  lack  of  action  of  the  flexor  carpi  ulnaris 
muscle,  the  movements  of  the  little  finger  are  impossible,  in  the  index,  middle 
and  ring  fingers,  the  proximal  phalanges  cannot  be  flexed,  the  distal  phalanges 
cannot  be  extended,  on  which  account,  when  the  trouble  lasts  long,  the  so-called 


.M 

glutaeus  maximus. 
(N.  glut.) 

N 

ischiadic™ 

M 

adduct.  magn.  (N.  obt.) 

M 

semitendin.  (N.  ischiad.) 

M. 

(N.  ischiad.) 

N. 

tibialis 

M.  gastrocnem.  (cap.  ext.). 
M.  gastrocnem.  (cap.  int.). 


M.  flex.  dig.  co 
M.  flexor  hallu 
N.  tibialis. 


Fig.  8. 
Motor  Points  of  the   Lower  Extremity,  Posterior  View. 


n.  long, 
loogus. 


"claw  hand"  is  produced  by  the  action  of  the  antagonists  (extension  at  the 
metacarpo-phalangeal — flexion  at  the  inter-phalangeal  joints).  The  paralysis 
of  the  interossei  also  causes  inability  to  spread  the  fingers  and  to  bring  them 
together  again.  On  account  of  the  preponderance  of  the  action  of  its  abductors 
over  that  of  the  paralyzed  adductor  pollicis,  the  thumb  is  continuously  sep- 
arated from  the  index  finger.  When  atrophy  sets  in,  the  sinking  in  of  the 
interosseous  spaces  and  the  thinning  of  the  hypothenar  region  are  particularly 
noticeable. 

h.  Paralysis  of  the  musculo-spiral  nerve  results  in  inability  to  extend  the 
hand  at  the  wrist-joint,  the  hand  hence  hangs  helpless  down  and  cannot  be 
raised  ("wrist  drop,"  see  Fig.  140.     When  the  seat  of  the  lesion  is  high  up, 


DISEASES    OF    THE    PERIPHERAL    NERVES 


21 


extension  at  the  elbow,  by  the  action  of  the  triceps  is  also  impossible;  of 
course  in  testing  this  function,  the  arm  must  be  put  in  a  position  eliminating 
the  action  of  gravity  on  the  elbow-joint.  Since  normally,  complete  flexion  of 
the  fingers  (as  in  squeezing  the  hand)  is  aided  by  extending  the  hand  at  the 
wrist,  in  musculo-spiral  paralysis  the  strength  of  the  flexors  of  the  fingers  is 
considerably  diminished,  unless  the  wrist  is  passively  extended  beforehand.  The 
thumb  is  held  in  a  position  of  opposition  and  falls  somewhat  forward.  The 
forearm  usually  takes  the  position  of  pronation.     The  paralysis  of  the  supinator 


Fio.  9. 

Paralysis  of  tin-  Serratus  on  the 
Right  Side  ("Winged  Scapula"). 


Paralysis  of  the  Suprascapular  Nerve  on  the  Right 
Side,  Atrophy  of  the  Supra-  and  Infraspinatus. 


[ongus  causes  a  weakening  of  flexion  at  the  elbow  joint,  also  when  this  move- 
ment is  attempted,  the  normally  prominent  outline  of  the  supinator  no  longer 
shows  up. 

i.  In  paralysis  of  the  anterior  crural  nerve,  the  thigh  cannot  be  flexed, 
the  leg  cannot  be  extended;  the  thigh  muscles  atrophy.  Walking  with  extension 
at  the  knee  is  indeed  possible,  in  that  the  patient  uses  his  leg  extended  by 
gravity  like  a  stilt,  but  lie  cannot  climb  the  stairs  with  it. 

/'.  Paralysis  of  the  tibialis  posting  nerve  prevents  flexion  of  the  toes,  the 
patient  cannot  raise  himself  on  his  toes  and  if  the  paralysis  lasts  long,  talipes 
calcaneus    may    develop.      The   toes    can    neither    be    spread    apart    nor   bent. 


22 


LECTURE    I 


Figs.  11  and  12. 

Ulnar   Paralysis    (trauma),  "Claw-hand." 


Fig.  13. 
Ulnar   Paralysis    (neuritis). 


Fig.   It. 
"Wrist  Drop"  in  Paralysis  of  the  Radial  Nerve; 
the  patient  is  unable  to  extend  the  hand  and 

fingers. 


DISEASES    OF    THE    PERIPHERAL    NERVES 


23 


Sometimes  there  develops  the  analogue  of  what  is  observed  in  the  hand  in  ulnar 
paralysis,  a  "claw  foot." 

k.  In  peroneal  paralysis  the  foot  hangs  in  the  so-called  "drop  foot"  posi- 
tion and  at  the  same  time  somewhat  s.upinated  (pes  equino-varus).  The  toes 
stand  flexed  at  the  interphalangeal  joint.  Since  the  patient  must  lift  his  foot 
abnormally  high  in  order  to  prevent  the  toes  dragging  on  the  ground,  there 
is  a  characteristic  gait,  which  Charcot  has  called  "steppage"  from  its  resem- 
blance to  the  action  of  an  English  thoroughbred  horse. 


Fin.  15. 
Traumatic  Paralysis  of  the  Peroneal  Nerve. 

The  tables  given  on  pages  14  and  15  will  furnish  sufficient  information  about 
the  paralyses  of  such  spinal  oerves  as  have  not  been  specially  described  here, 
and  also  with  regard  to  cases  in  which  the  territories  of  the  nerves  mentioned 
are  only  partially  affected.       ' 

'•}.     Areas  of  Distribution  of  the  Spinal  Sensory  Nerves  ami  Peripheral 
Anesthesias 


Figs.  16  and  IT  show  the  skin  areas  of  the  different  sensory  nerve  trunks 
and    their   blanches.      It    must,    however,   be    remarked    that    the   distribution    of 


24 


LECTURE    I 


single  skin  nerves  is  quite  variable;  the  limits  can  show  considerable  shifting 
in  favor  of  the  one  or  of  the  other  nerve.  It  is  to  be  emphasized  also  that  in 
the  etiologically  most  different  varieties  of  interference  with  conduction,  even 


Fig.   17. 


when  they  affect  not  purely  motor,  but  "mixed"  nerves,  sensation  may  remain 
very  little  disturbed  or  even  intact.  As  far  as  they  refer  to  the  types  of 
peripheral  paralyses  already  considered,  the  sensory  disturbances  are  as- 
follows : 


DISEASES    OF    THE    PERIPHERAL    NERVES 


2.5 


Total  brachial  plexus  pa- 
ralysis 

Upper  plexus  paralysis 

Lower  plexus  paralysis 

Nervus  thoracic,  long. 

Nervus  suprascapularis 

Nervus  axillaris  (circum- 
flex) 

Nervus  musculo-cutan. 
Nervus  medianus 


Nervus  ulnaris 

Nervus   radialis    (muscu- 
lo-spiral) 


Nervus  cruralis 
Nervus  tibialis 
Nervus  peroneus 


Disturbances  of  Sensation 

Usually  present;  the  inside  of  the  arm  is  almost  always  unaffected, 
otherwise  the  distribution  varies  greatly 

Less  frequent:  as  rule  located  on  the  outside  of  the  arm 

Are   found   in   the   majority   of   cases,   particularly    in    the    ulnar 
distribution,  on  the  inner  side  of  both  arm  and  forearm 

None 

None 

S etimes  on   the  outside  of  the  arm    (see   Figs.   16  and   17) 


On  the  outside  of  the  forearm  (nervus  eutan.  lateral)  (see  Figs. 
l(i   and   17) 

In  typical  cases  on  the  radial  parts  of  the  palm  (as  in  Fig.  17) ; 
also  dorsally  on  the  terminal  phalanges  of  fingers  II,  III  and  IV 
(as  in  Fig.  Hi);  the  dorsum,  however,  often  remains  unaffected, 
and  also  the  palmar  area  may  be  only  partially  involved;  occa- 
sionally no  sensory  disturbances  at  all 

Almost  always  present;  in  the  ulnar  part  of  palm  and  dorsum 
of  the  hand  they  occupy  the  region  indicated  in  Figs.  16  and  17 

Often  absent;  most  frequent,  however,  on  the  radial  half  of  the 
back  of  the  hand;  only  exceptionally  an-  the  territories  of  the 
cutaneous  post,  infer,  and  cutaneus  post.  sup.  involved  (see 
Fig.   16) 

Sometimes  sensory  disturbances  corresponding  to  the  anas  marked 
"Our."  and  "Saph."  in  Fig.  17 

Sometimes  sensory  disturbances  in  the  areas  marked  "Cut.  crur. 
post,  med.,"  "Plant,  lat."  and  "Plant,  tned."  in  Fig.  16 

Chiefly  sensory  disturbances  in  the  areas  marked  "Cut.  peron." 
and  "Per."  in  Figs.  16  and  17.  Their  extent  varies  greatly. 
however 


LECTURE  II 
Diseases  of  the  Peripheral  Nerves 

A.     Disturbances  of  Conduction  (Continued) 

SPECIAL   SYMPTOMATOLOGY  OF  THE  DEFECTS  OF  FUNCTION 
IN  THE  DIFFERENT  PERIPHERAL  NERVES 

II.     The  Cranial  Nerves  . 

In  the  first  lecture,  after  we  had  considered  the  etiology,  the  pathogenesis 
and  the  pathological  anatomy  of  disturbances  of  conduction  in  the  peripheral 
nerves,  we  took  up  the  general  symptomatology  of  those  pathological  condi- 
tions and  finally  considered  the  defects  of  function  which  occur  in  lesions  of 
the  spinal  nerves.  We  must  now  discuss  in  their  proper  sequence  the  symptoms 
of  defective  function  in  the  cranial  nerves  when  diseased. 

1.  The  Olfactory. — Loss  of  sense  of  smell  is  called  anosmia;  its  reduction 
hyposmia.  To  test  the  sense  of  smell  only  such  reagents  should  be  used  as 
do  not  (for  example  like  acetic  acid  or  ammonia)  act  as  irritants  to  the 
branches  of  the  trigeminus  which  are  distributed  to  the  mucous  membrane  of 
the  nose,  e.g.,  turpentine,  oil  of  rose,  tar,  benzene,  tinct.  A'alerian. 

2.  The  Optic. — The  symptoms  of  defect  upon  the  part  of  the  optic  nerve 
are  manifold  and  for  their  determination  and  clinical  interpretation  we  are 
largely  dependent  upon  the  assistance  of  the  ophthalmologist.  Hence  we  can 
give  only  a  summary  account  of  them  here.  Total  destruction  of  one  optic 
nerve  causes  complete  blindness  in  the.  corresponding  eye  (amaurosis)  ;  hence 
throwing  a  beam  of  light  into  the  amaurotic  eye  can  no  longer  cause  reflex 
contraction  of  the  pupil.  This  can,  however,  be  provoked  by  the  action  of 
light  upon  the  sound  eye.  Slight  alterations  of  the  optic  nerve  cause  dimness 
of  vision,  amblyopia. 

Partial,  lacunar  defects  in  the  visual  field  are  called  scotomata,  according  to 
their  location,  central  or  peripheral  scotomata.  When  the  perception  of  single 
colors  is  lost  in  a  limited  portion  of  the  field,  we  speak  of  color  scotoma.  If  the 
radii  of  the  field  of  vision  of  one  eye  are  comparatively  evenly  shortened,  we  have 
concentric  narrowing  of  the  visual  field.  So  far  the  symptoms  of  defect  which 
occur  in  peripheral  diseases  of  the  optic  nerve;  half-sided  loss  of  vision,  the 
so-called  hemianopsia  is  found  only  in  lesions  of  the  visual  tract  back  of  the 
chiasma;  hence  it  will  not  be  discussed  until  we  come  to  diseases  of  the  brain. 
Of  all  the  cranial  nerves,  the  optic  occupies  a  unique  position,  in  that  it  alone 
is  accessible  to  direct  inspection  by  the  aid  of  the  ophthalmoscope.     Three  sorts 

26 


DISEASES    OF    THE    PERIPHERAL    NERVES  27 

of  pictures  may  be  presented  to  us  in  disturbance  of  conduction  in  the  peripheral 
course  of  the  optic  fibers.  If  the  optic  nerve  is  affected  by  a  tumor  or  an 
injury,  it  undergoes  ""simple  atrophy";  the  papilla  becomes  paler,  eventually 
very  white  and  at  the  same  time  there  iscupping  (atrophic  excavation)  and  the 
gray  points  of  the  lamina  cribrosa  are  specially  prominent ;  from  the  surround- 
ing retina  it  is  abnormally  sharply  delimited,  the  finer  blood  vessels  of  the  nerve 
head  disappear  more  and  more.  "Optic  neuritis,"  on  the  other  hand,  pro- 
vided that  the  papilla  is  also  affected,  which  is  not  necessarily  always  the  case,*- 
gives  the  following  ophthalmoscopic  changes :  The  papilla  is  more  or  less 
prominent  in  the  eye  ground,  it  is  reddish  gray  in  color,  sometimes  appears 


Fig.  18. 
Left-sided   Oculomotor   Paralysis.      Ptosis. 


mottled,  its  boundaries  are  not  clear,  its  diameter  appears  increased,  its  reins 
are  swollen  and  tortuous,  the  arteries,  on  the  contrary,  narrow. 

As  a  result  of  intense  and  advanced  optic  neuritis,  finally,  there  is  a  third 
pathological  ophthalmoscopic  picture,  the  so-called  "inflammatory  atrophy." 
In  this  the  papilla  is  at  the  start  gray  white  and  shows  indistinct  outlines  i 
later  it  becomes,  like  in  simple  atrophy,  very  white  with  sharply  defined  bounda- 
ries, but  nevertheless  appears  irregular  and  shrunken;  also  the  lamina  cribrosa 
does  not  show  up  and  the  neighboring  choroid  in  many  cases  presents  irregular 
decolorization. 

.'{.  The  Oculomotor  Nerve.     The  third  cranial   nerve  innervates  both   the 


"When  inflammation  ot  tl»-   papilla  is  absent  or  very  slight,  we  speak   of  "retrobulbar 

neuritis." 


28 


LECTURE    II 


external  and  the  internal  muscles  of  the  eye.  Namely,  to  begin  with  the  first, 
the  levator  palpebrs,  which  raises  the  upper  lid,  the  rectus  internus,  which  draws 
the  eyeball  inward,  the  rectus  superior  and  rectus  inferior,  which  draw  it  re- 
spectively upward  and  downward,  and  the  inferior  oblique,  whose  action  con- 
sists in  rolling  the  ball  upon  its  axis  so  that  the  lower  portion  of  its  periphery 
is  turned  inward  toward  the  nasal  border  of  the  orbit.  The  internal  muscles 
innervated  by  the  oculomotor  are.  the  ciliary  muscle,  through  whose  contraction 
the  zonula  of  the  lens  relaxes  (from  which  greater  convexity  of  the  lens  and 


Fig.  19. 
Paralysis  of  the  Internal  Rectus  on  the  Right  Side   (Fracture  at  the  Base  of  the  SkulJ). 


accommodation  for  near  objects  results)  and  the  ring  fibers  of  the  iris,  the 
sphincter  pupilla?.  In  complete  paralysis  of  the  oculomotor,  the  upper  lid 
hangs  flaccid  (ptosis)  (see  Fig.  18)  and  the  eye  is  drawn  outward  and  down- 
ward, this  through  the  unopposed  action  of  the  rectus  externus  and  superior 
oblique  muscles,  which  are  innervated  by  the  other  eye  muscle  nerves  (the 
abducens  and  trochlearis).  Besides  this,  the  pupil  is  dilated  and  the  eye  remains 
in  a  position  of  accommodation  for  distance  (paralysis  of  the  sphincter  pupillre 
and  ciliary  muscles).  When  the  disturbance  of  conduction  in  the  oculomotor 
nerve  is  caused  by  progressive  processes  at  the  base  of  the  brain,  for  example, 
tumors,  ptosis  is  usually  the  first  symptom.  The  distinction  between  an  oculo- 
motor paralysis  from  lesion  of  the  nerve  itself  and  one  due  to  disease  of  its 


DISEASES    OF    THE    PERIPHERAL    NERVES  29 

nucleus  in  the  brain  axis  is  not  always  easy ;  it  is  well  to  remark,  however,  that 
in  nuclear  paralyses  the  internal  muscles  of  the  eye  usually  escape.  If  on  the 
contrary  these  alone  are  involved,  we  speak  of  "ophthalmoplegia  interna." 

4.  Trochlearis. — This  nerve  supplies  exclusively  the  superior  oblique  mus- 
cle, which  as  antagonist  of  the  inferior  oblique,  rolls  the  eyeball  inward,  its 
upper  periphery  toward  the  nasal  border  of  the  orbit. 

5.  The  Trigeminus. — The  trigeminus  possesses  a  large  sensory,  a  smaller 
motor  portion,  of  which  the  first  divides  into  three  large  divisions,  the  Ramus 
ophthalmicus,  R.  maxillaris  and  R.  mandibularis.  The  following  table  presents 
the  sensory  distribution  of  these  three  branches. 

Ramus  primus    (I),  seu  Ramus  secuxuus   (II),  seu  Ramus  tertius  (III),  seu 

OPHTHALMICUS  MAXILLARIS  MAXDIBULARIS 

(a)  The  skin  area  marked  1  in    (a)   The   skin   area   2,   in    Fig.    (a)    The   skin    area   3,   in   Fig. 
Fig.   20  20  20 

(b)  Conjunctiva,     cornea      and    (l>)    The  mucous  membrane  of   (b)   The  mucous  membrane  of 
iris  the    antrum    and    the    lower       the    cheeks,   the   lower   jaw, 

parts    of    the    nose  the   floor   of   the  mouth,   the 

tongue 

(c)  The   mucous   membrane   of   (c)  The  mucous  membranes  of  (c)   The  lower  teeth 
the    frontal    sinuses    and    the       the   upper  jaw   and   of   the 

upper  parts  of  the  nose  palate  to  the  palatopharyn- 

geal arch 
(d)   The  upper  teeth 

In  total  or  partial  destruction  of  one  of  the  three  branches,  there  is  of 
course  anesthesia  or  hypoesthesia  in  the  corresponding  parts.  Further,  in  lesion 
of  the  ophthalmic  branch,  there  is  loss  of  the  conjunctival  and  corneal  reflex 
(closing  of  the  eye  on  touching  the  conjunctiva  or  cornea  with  a  blunt  object, 
for  instance,  the  head  of  a  pin,  also  of  the  reflex  of  sneezing  produced  by 
tickling  the  upper  part  of  the  nasal  mucous  membrane.  In  lesion  of  the  max- 
illary branch  the  sneezing  reflex  is  absent  when  the  lower  part  of  the  nasal 
mucosa  is  irritated,  as  is  also  the  palate  reflex,  that  is  the  movements  of  swallow- 
ing or  of  retching  produced  by  tickling  the  soft  palate. 

It  is  to  be  remarked  here,  however,  that  all  of  these  three  reflexes  are 
inconstant  and  subject  to  great  individual  variations.  Hence  only  the  absence 
of  these  phenomena  is  of  diagnostic  importance.  It  is  well  to  recall,  too,  that 
the  sensation  of  pricking  perceived  on  inhaling  ammonia  or  acetic  acid  is 
produced  not  through  the  olfactory  nerve  hut  by  irritation  of  sensory  ter- 
minations of  the  trigeminus. 

If  the  trigeminus  is  out  of  function  these  do  not  occur,  neither  do  the  reflex 
Bymptoms  produced  through  if  (watering  of  the  eves,  changes  in  the  pulse, 
arrest  of  respiration). 

Besides  conducting  common  sensation  the  trigeminus  contains  also  fibers  for 
special  sense.  The  mandibularis,  through  one  of  its  branches,  the  lingualis 
collects  the  taste  fibers  from  the  anterior  two-thirds  of  the  tongue;  these  pro- 
ceed then  through  the  chorda  tympani  to  the  facial  trunk,  to  return  again  to 
the  trigeminus  after  they  have  run  some  distance  in  the  facial.     Since  disturb- 


30 


LECTURE    II 


ances  of  taste  (ageusia,  hypogeusia)  occur  most  commonly  in  facial  paralysis, 
we  will  postpone  the  discussion  of  the  methods  of  testing  taste,  until  we  come 
to  speak  of  the  facial  nerve.  The  motor  trigeminus  fibers  are  included  in  the 
third  branch  and  are  distributed  to  the  muscles  of  mastication,  the  anterior 
belly  of  the  digastric,  the  mylohyoid,  the  tensor  tympani  and  the  tensor  palati 
muscles.  Unilateral  paralysis  of  the  muscles  of  mastication  (masseter,  tem- 
poral, pterygoids)  is  called  monoplegia  masti- 
catoria.  In  this,  lateral  movement  of  the 
lower  jaw  is  only  possible  toward  the  para- 
lyzed side-,  since  only  on  the  sound  side  are 
the  pterygoids  still  able  to  contract.  By 
palpation  the  absence  of  contraction  in  the 
masseter  and  temporal  of  the  affected  side 
can  also  be  noted.  In  diplegia  masticatoria, 
the  lower  jaw  drops  and  all  lateral  motion  is 
impossible.  Further,  there  is  no  jaw  reflex — 
the  contraction  of  the  masseters  on  striking 
upon  the  finger  laid  upon  the  lower  row  of 
teeth,  which  can  be  obtained  in  most  normal 
individuals. 

The  paralysis  of  the  anterior  belly  of  the 
digastric  and  of  the  mylohyoid,  sometimes 
produces  a  sense  of  relaxation  on  palpation 
in  the  muscles  of  the  floor  of  the  mouth  on 
the  paralyzed  side;  we  have  little  informa- 
tion as  to  the  symptoms  of  loss  of  function 
on  the  part  of  the  tensor  tympani  and  the 
tensor  palati.  It  is  certain  that  in  most 
cases  they  remain  latent ;  here  and  there, 
however,  there  appear  to  be  anomalies  in  the 
position  of  the  palatopharyngeal  arch  and 
poor  hearing  for  low  tones. 

Besides  the  motor  and  sensory  fibers 
mentioned,  immediately  after  their  exit 
from  the  skull  all  of  the  three  divisions  of  the  trigeminus  have  sympathetic 
fibers  added  to  them,  and  these  fibers  accompany  these  divisions  and  their 
branches  in  their  further  course.  The  meeting  points  of  these  sympathetic 
fibers  (they  arise  altogether  from  the  plexus  accompanying  the  head  arteries) 
with  trigeminus  neurones,  are  at  certain  nodes  or  ganglia  (ganglion  ciliaris, 
sphenopalatinum,  oticum,  linguale).  Hence,  under  some  circumstances,  a  lesion 
of  the  first  division  can  have  as  a  result  a  narrowing  of  the  palpebral  fissure  and 
a  myosis  of  the  pupil  on  account  of  paralysis  of  the  tarsalis  superior  and 
dilator  pupilla?  muscles  innervated  by  the  sympathetic,  a  lesion  of  the  second 
division,  by  paralysis  of  the  orbital  muscle  at  the  base  of  the  orbit,  an  "enoph- 
thalmus,"  that  is,  a  sinking  in  of  the  eyeball.  All  of  the  three  branches  carry 
sympathetic  fibers  to  the  blood  vessels  and  sweat  glands,  on  which  account  in 
interruption  of  conduction  we  almost  always  find  in  the  anesthetic  parts  heat 


The    Sensory    Nerve    Supply    c 
Head 

Hatched:    Trigeminus 

1.  The  ophthalmic  branch 

2.  The   maxillary   branch 

3.  The   mandibular   branch 


White:   Vagus 

v.  Nervus   auricularis 


,-agi 


Black:    Cervical    nerves 
O,  Great  occipital  nerve 
o,  Lesser   occipital   nerve 
A,  Great  auricular  nerve 


DISEASES    OF    THE    PERIPHERAL    NERVES  31 

and  redness  (in  fresh  cases)  or  cyanosis  and  coldness  (in  older  cases),  as  well 
as  anidrosis.  Other  neurones  originally  sympathetic,  but  running  in  the 
trigeminus,  which  must  be  mentioned,  are  fibers  regulating  the  secretion  of 
the  nasal  mucus,  contained  in  the  ophthalmic  and  maxillary  divisions.  Upon 
their  failure  to  functionate  depends  the  abnormal  dryness  of  the  nasal  mucosa 
which  occurs  in  paralyses  of  these  nerves  and  leads  secondarily  to  reduction  of 
the  sense  of  smell.  Finally,  there  are  other  secretory  fibers  coming  from  another 
cranial  nerve,  the  facial,  the  already  mentioned  lingual  nerve  containing  those 
for  the  salivary  secretion,  the  lacrymal  nerve  (a  branch  of  the  ophthalmic) 
those  for  the  tear  secretion.  Hence  these  secretions  can  sometimes  be  affected, 
in  troubles  of  the  fifth  nerve. 

6.   The  Abducens. — To  the  sixth  nerve  belongs  the  motor  innervation  of 


Fig.  21. 
Bilateral  Abducens  Paralysis. 

the  external  rectus  muscle  which  turns  the  eye  about  a  vertical  axis  outward, 
abducts  it.  Isolated  abducens  paralysis  has  Inner  as  a  result  a  deviation  of 
the  affected  eye  inward  (see  Fig.  21).  Winn  the  abducens  is  affected,  together 
with  the  oculomotorius  and  trochlearis  (ophthalmoplegia  totalis),  the  eve 
remains  immovable,  directed  to  the  front. 

7.  The  Facial. — This  nerve  supplies  all  the  muscles  of  the  lace  (including 
the  buccinator,  excepting  the  levator  palpebral  superioris,  which  is  innervated 
Dy    the    oculomotor)     from    the    frontal    muscle    to    the    pla  t  vsma    mvoides,    and 

besides  these,  the  stylohoid,  the  posterior  belly  of  the  digasl  ric  and  the  stapedius. 
The  failure  of  the  motor  function  of  the  facial  nerve  produces  the  clinical 
picture  of  facial  paralysis  (<>r  prosoplegia)  which  when  unilateral  is  denomi 
Dated  monoplegia  facialis  (see  Figs.  '2'2  and  28),  when  bilateral,  diplegia  facialis. 

It  is  also  called  Bell's  paralysis.  The  paralyzed  half  of  the  face  is  devoid  of 
the  mimic  movements,  masklike,  immovable,  expressionless;  the  naso  labial  fold 


32  LECTURE    II 

is  obliterated,  the  forehead  cannot  be  wrinkled,  the  eye— on  account  of  the 
paralysis  of  the  orbicularis  palpebrarum — cannot  be  closed  (lagophthalmus), 
the  angle  of  the  mouth  hangs  down.  The  falling  downward  of  the  back  portion 
of  the  tongue  betrays  the  paralysis  of  the  stylohoid  and  the  posterior  belly  of 
the  digastric,  an  abnormal  acuteness  of  hearing  and  a  sensitiveness  to  deep 
tones  (oxyakoia,  hyperacusis),  that  of  the  stapedius  whose  function  it  is  to 
close  the  fenestra  ovalis  of  the  drum  cavity  by  drawing  on  the  stapes.  From  the 
neighborhood  of  its  origin  also,  the  facial  trunk  carries  along  with  it  other  sorts 
of  centrifugal  fibers,  namely  those  for  the  tear  secretion  and  those  for  the 
.salivary  secretion;  both  of  these  sets  of  fibers  as  we  have  already  heard,  enter 


Fig.  22. 
Left-Sided  Peripheral  Facial  Paralysis. 

the  trigeminal  nerve,  while  on  the  other  hand  the  taste  fibers  from  the  anterior 
two-thirds  of  the  tongue  leave  the  trigeminus  for  some  distance  and  join  the 
facial.  Fig.  24*  will  bring  before  you  the  anatomical  relations  of  this  somewhat 
complicated  exchange  of  fibers.  They  contribute  chiefly  to  the  fact  that 
according  to  where  a  break  in  the  conduction  in  the  peripheral  course  of  the 
facial  has  occurred  different  clinical  symptoms  develop.  I  have  presented  these 
in  tabular  form  on  page  33. 

Testing  of  sense  of  taste  on  the  tongue  is  carried  out  in  the  following 
manner.  The  subject  is  instructed  to  tell  the  taste  of  the  solutions  which 
are  applied  to  his  outstretched  tongue  without  drawing  it  back  into  his  mouth 
again.  These  precautions  are  to  prevent  the  falsification  of  the  results  by  their 
perception  by  the  mucous  membrane  of  other  parts  of  the  mouth  which  are 
innervated  by  the  glossopharyngeal.     A  card  bearing  the  words  "bitter,  sour, 


DISEASES    OF    THE    PERIPHERAL    NERVES  33 

salty,  sweet,"  is  placed  before  the  patient  and  he  is  asked  to  indicate  upon  it 
what  he  tastes  as  his  tongue  is  touched  successively  by  cotton  brushes  wet  with 
'  tinct.  gentian,  vinegar,  salt  solution,  and  syrup.  After  each  test  the  tongue 
must  be  washed  off  with  a  wisp  of  wet  cotton.  The  eyes  are  best  covered  during 
these  tests. 

BREACHES  OF  CONDUCTION'   IX   THE   FACIAL  NERVE. 

a.  Outside  of  the  cranial  cavity. 

Almost  exclusively  monoplegia  facialis  (Exception:  facial  paralysis  from 
double  otitis). 

a.  Distal  to  the  origin  of  the  chorda  tympani. 

(Area  1,  Fig.  24.)  Symptoms:  paralysis  of  the  facial  muscles;  if  the 
lesion  is  very  far  toward  the  periphery,  that  is  beyond  the  pes  anse- 
rinus,  some  of  the  branches  may  escape. 

b.  In  the  Fallopian  canal,  between  the  chorda  and  the  origin  of  the 
stapedius  (Fig.  24,  2).  Symptoms:  facial  paralysis;  loss  of  taste  on 
the  anterior  two-thirds  of  the  tongue;  salivary  secretion  altered. 

e.  In  the  Fallopian  canal  between  the  origin  of  the  stapedius  and  the 
geniculate  ganglion  (Fig.  24,  3).  Symptoms:  facial  paralysis,  loss 
of  taste  on  the  anterior  two-thirds  of  the  tongue,  salivary  secretion 
altered,  hyperacusis. 

d.  Between  the  meatus  acusticus  interims  and  the  geniculate  ganglion 
(Fig.  24,  4).  Symptoms:  facial  paralysis,  no  taste  disturbance,  alter- 
ation of  the  salivary  secretion,  often  nerve  deafness  (see  below) 
through  involvement  of  the  auditory,  only  when  this  is  not  present, 
hyperacusis,  loss  of  the  affective  and  reflex  tear  secretion. 

b.  Within    the    cranial    cavity — "basal"    lesions    of   the    facial.        Not     infre- 

quently diplegia  facialis  (basal  gummatous  meningitis).  Symptoms: 
as  above  under  J  generally  involvement  of  a  number  of  basal  nerve 
roots  (al)dueens,  glossopharyngeus,  vagus,  accessorius,  hypoglossus) ; 
general  brain  symptoms  (vertigo,  vomiting,  headache). 

8.  The  Audit  or  i/. — The  eighth  cranial  nerve  is  made  up  of  two  portions 
each  of  differenl  function:  the  nerve  of  hearing  proper,  the  cochlear,  and  the 
nerve  of  orientation  in  space,  the  vestibular.  The  firs!  stands  in  connection 
with  the  cerebrum,  the  latter  with  the  cerebellum,  the  organ  for  the  preserved  ion 
of  the  equilibrium. 

Loss  of  function  of  the  cochlear  nerve  produces  impairment  of  hearing 
to  complete  deafness  (hvpacllsis  or  anacusis ) .  Since,  however,  these  disturb- 
gmces  may  also  be  produced  by  affections  of  the  sound-conducting  apparatus, 
of  the  middle  or  of  the  outer  ear,*   the  chief  characteristics  of  "hardness  of 

*  A  lesion  in  the  perceptive  apparatus  in  the  cochlea  of  the  lahyrinth  (thai  is.  In  the 
organ  of  Corti)  gives  the  same  symptoms  as  a  breach  of  conduction  in  the  cochlear  nerve. 


34 


LECTURE    II 


hearing  or  deafness  of  nerve  origin"  must  be  considered.  These  are:  1.  The 
reduction  or  loss  of  hearing  through  bone  conduction.  2.  Partial  loss  of  per- 
ception for  the  notes  of  the  scale. 

The  first  of  these  phenomena  is  determined,  a,  by  Schwabach's  test.  In  this 
the  time  during  which  a  vibrating  tuning-fork  placed  upon  the  parietal  bone, 
the  teeth,  or  the  mastoid  process,  can  be  heard,  on  the  one  hand,  by  the  person 
under  examination,  and  on  the  other  by  the  normally  hearing  examiner,  is  com- 
pared. In  impairment  of  hearing  from  nerve  lesion,  there  is  shortening  of  the 
period  during  which  the  sound  can  be  perceived  through  bone  conduction;  in 


Fig.  33. 
Left-Sided  Peripheral  Facial  Paralysis,  Upon  Attempt  to  Close  Both  Eyes. 


nerve  deafness  bone  conduction  may  be  lost.  In  middle  ear  affections  on  the 
contrary  the  tuning-fork  applied  to  the  bone  is  heard  longer  than  normal. 
This  method  is  naturally  only  applicable  in  bilateral  disturbances  of  hearing. 

b.  By  "Rinne's  test." — In  this,  a  vibrating  tuning-fork  is  placed  upon  the 
mastoid  process;  when  the  patient  ceases  to  hear  it  there  (that  is,  bone  con- 
duction is  no  longer  perceived),  the  fork  is  transferred  to  opposite  the  ear.  The 
normal  person  now  hears  the  sound  anew  and  this  is  denominated  a  positive 
result  ("Rhine  positive").  In  disease  of  the  sound-conducting  apparatus  (where 
the  perception  through  air  conduction  is  diminished,  but  through  bone  conduc- 
tion unaltered)  in  the  second  part  of  the  test,  sound  is  no  longer  perceived,  it 
is   negative. 

In  nerve  "hypoacusis"  on  the  contrary,  there  is,  as  a  rule,  "positive  Rhine" 


DISEASES    OF    THE    PERIPHERAL    NERVES 


35 


provided,  of  course,  that  there  is  not  a  high  degree  of  defect  of  hearing.  In 
this  latter  case,  as  in  nerve  deafness,  hearing  through  air  conduction  is  con- 
siderably reduced  or  lost. 

c.  Bv  "Weber's  test."  If  in  a  normal  individual  a  vibrating  tuning-fork  is 
placed  on  the  vertex  it  is  heard  in  both  ears;  if  now  one  ear  is  plugged  up,  the 
sound  is  "lateralized"  on  the  side  upon  which  air  conduction  is  in  this  manner 


To   the    N.    lacrymal.    trige 


G.  =  Gangl.    genl 


Fig.  34. 
Diagram  of  the  Different  Categories  of   Fibers  in  tlie  Facial  Nerve. 

interrupted.  This  "lateralization"  of  the  sound  occurs  spontaneously  on  the 
side  of  impaired  hearing  in  persons  with  diseases  of  the  sound  conducting  ap- 
paratus ("positive  Weber"),  Wmle  the  person  with  nerve  deafness  lateralizes  t<> 
the  -.111111(1  side  ("negative  Weber"). 

Defects  in  the  perception  of  the  notes  of  the  scale  .ire  tested  by  the  so-called 
Galtoris  whistle.  Deafness  for  the  upper  notes  of  the  scale  in  particularly 
characteristic  of  affections  of  auditory  nerve  tracts  as  opposed  to  those  of  the 
middle  and  outer  ear.  In  "nervous  hardness  of  hearing"  the  perception  of 
words  witli  sharp  consonants  and  clear  vowels  (as  sheriff,  swagger,  fish,  wasp), 


36  LECTURE    II 

suffers  especially,  while  those  with  dull  consonants  and  vowels  (as  brooder, 
hunger,  ore,  worm*)  are  much  better  understood.  The  opposite  is  the  case 
in  non-nervous  hypoacusis. 

The  diagnosis  of  nerve  hardness  of  hearing  and  deafness  must  be  made 
in  general  by  exclusion,  after  examination  of  the  drum  membrane,  when  the 
hearing  is  not  improved  after  Pollitzerization  of  the  ear,  etc.  As  to  the  value 
of  that  peculiar  phenomenon,  the  so-called  paracusis  of  Willis  for  the  diagnosis 
of  nerve  deafness,  opinions  arc  divided.  Many  otologists  are  of  the  opinion 
that  nerve  deafness  paradoxically  makes  itself  less  felt  in  a  noisy  place  (in  a 
railway  car)  than  in  a  quiet  one;  according  to  others  still,  in  middle  ear  disease 
"Paracusis  Willisii"  can  also  occur. 

The  vestibular  nerve  transmits  to  the  cerebellum  information  as  to  the 
position  in  space  of  the  body,  especially  of  the  head.  The  sensory  epithelium 
of  the  ampulla1,  the  utricle  and  the  saccule  of  the  labyrinth  are  stimulated  to 
function  through  the  hydrostatic  relations  of  the  fluid  in  the  semicircular  canals 
(which  are  arranged  according  to  the  three  planes  of  space).  If  now  there 
is  a  contradiction  between  the  condition  of  stimulation  and  the  actual  position 
in  space  of  the  body,  the  resulting  confusion  gives  to  the  patient  the  illusion  of 
rotary  movements,  either  of  his  own  body  or  of  surrounding  objects,  which  we 
denominate  rotary  vertigo.  The  last  can  arise  from  interruption  of  conduction 
in  the  vestibular  nerve  and  then  shows  itself  sometimes  by  moderate  uncertainty 
of  gait  and  difficulty  in  turning,  as  well  as  by  holding  the  head  stiffly. 

We  denominate  "'galvanic  vertigo,"  a  manifestation  which  appears,  when 
in  the  person  under  investigation,  a  constant  current  of  gradually  increasing 
intensity  is  conducted  through  the  head  at  the  level  of  the  ears.  When  a  cur- 
rent strength  of  about  5  to  6  milleamperes  is  reached,  the  normal  person  has  the 
feeling  that  he  is  going  to  fall  toward  the  side  on  which  the  positive  pole  is, 
and  usually  inclines  his  head  to  that  side.  If  one  vestibular  nerve  is  out  of 
function,  however,  in  many  cases  the  patient  in  this  experiment  has  each  time  the 
feeling  that  he  is  sinking  toward  the  side  of  the  injured  nerve  regardless  of  the 
position  of  the  anode  or  the  cathode. 

More  applicable  to  the  clinical  investigation  of  the  vestibular  apparatus  is 
Barony's  test,  that  is,  the  examination  for  caloric  nystagmus.  Each  vestibular 
apparatus  is  in  anatomical  and  physiological  connection  with  the  eye  muscles 
through  the  fasciculus  longitudinalis  posterior,  on  account  of  which,  irritation 
of  the  ear  by  syringing  with  hot  or  cold  water  produces  rhythmical  horizontal 
movements  of  the  eye.  (Probably  through  the  differences  of  temperature  there 
are  produced  movements  of  the  endolymph,  which  irritate  the  nerve  structures 
of  the  labyrinth.)  In  healthy  persons,  the  syringing  of  the  ear  with  cold  water 
produces  nystagmus  toward  the  opposite  side;  if  hot  water  is  used,  the 
nystagmus  on  the  contrary  is  toward  the  side  of  the  syringed  ear.  If  the 
vestibular  nerve  is  paralyzed,  no  caloric  reaction  can  be  produced  from  the 
corresponding  ear.  The  healthy  side  generally  reacts  normally,  though  occa- 
sionally irritability  on  this  side  is  also  somewhat  reduced. 

9.   The  Glossopharyngeal  Nerve. — This  nerve  is  mainly  sensory?  but  has  a 

*  For  the  German  words  given  by  the  author,  English  words  of  as  similar  sound  as 
possible  have  been   substituted. — Translator. 


DISEASES    OF    THE    PERIPHERAL    NERVES  37 

motor  division  which  supplies  the  stylopharyngeus  muscle,  an  elevator  of  the 
pharynx.  The  glossopharyngeal  transmits  sensory  impressions  from  the  upper- 
most part  of  the  pharynx  and  from  the  middle  ear,  as  well  as  taste  perceptions 
from  the  palate  and  from  the  posterior  third  of  the  tongue.  On  this  account, 
ageusia  in  the  last-mentioned  region  is  the  most  important  symptom  of  its 
loss  of  function ;  also  an  anesthesia  of  the  pharynx  can  be  recognized,  as  well 
as  loss  of  the  pharyngeal  reflex.  This  last,  however  (in  correspondence  with 
what  was  mentioned  in  connection  with  the  palate  reflex),  can  only  be  consid- 
ered as  a  sign  of  breach  of  conduction  in  the  glossopharyngeal  when  there  is  a 
difference  between  the  reflexes  on  the  two  sides.  The  stylo-pharyngeus  muscle, 
as  elevator  of  the  pharynx — in  which  function  it  unites  itself  with  the  pharyngo- 
palatine,  supplied  by  the  vagus — is  of  too  little  importance  for  motor  symptoms 
to  occur  from  its  elimination  alone. 

10.  The  Vagus  (or  Pncumogastric)  Nerve. — The  vagus  is  a  mixed  nerve. 
On  the  one  hand  it  supplies  the  muscles  of  the  palate,  pharynx,  larynx,  trachea 
and  bronchi,  as  well  as  those  of  the  oesophagus,  stomach  and  small  intestine, 
with  motor  fibers,  and  carries  inhibitory  fibers  for  the  heart  and  vasomotor 
fibers  for  numerous  vessels ;  on  the  other,  it  is  the  sensory  nreve  for  the  dura 
mater,  the  external  auditory  canal,*  the  lower  portion  of  the  pharynx,  the 
Larynx,  the  trachea  and  bronchi,  the  oesophagus  and  the  stomach.  Total  dou- 
ble paralysis  of  the  vagus  has  no  symptomatology,  since  it  is  incompatible 
with  further  existence.  On  the  other  hand,  a  partial  double,  or  a  total  single 
vagus  paralysis,  can  occur  clinically.  In  the  last  case  there  is  a  unilateral 
paralysis  of  the  uvula,  pharynx  and  larynx.  One-half  of  the  velum  palati 
hangs  flaccid  down,  on  which  account  the  voice  becomes  nasal.  One  vocal  cord 
is  immovable  in  the  middle  or  the  cadaveric  position,  since  both  the  closing 
and  the  opening  muscles  of  the  glottis  are  put  out  of  action.  Still,  the  voice 
may  remain  normal  through  compensatory  overaction  of  the  other  vocal  cord. 
Usually,  indeed,  it  is  somewhat  hoarse,  changing  to  falsetto.  On  the  other 
hand,  disturbances  in  swallowing  are  almost  always  minimal,  since  hemi- 
pharyngoplegia,  on  account  of  the  mutual  interlacing  of  the  muscular  fibers 
of  the  pharynx,  is  without  great  functional  importance.  An  increased  fre- 
quency of  the  heart  beat — a  tachycardia — has  very  seldom  been  observed  in 
unilateral  loss  of  function  of  the  vagus ;  tin-  same  thing  is  true  with  regard  to 
disturbances  of  respiration  (in  the  sense  of  slowing  and  irregularity  of  the 
breathing).  In  complete  paralysis  of  the  vagus,  only  a  few  of  the  .above- 
related  symptoms  are  present,  sometimes  these,  indeed,  are  only  partially  de- 
veloped. So,  instead  of  total  paralysis  of  the  vocal  cords,  there  is  only  paraly- 
sis of  the  posterior  cricoarytenoid,  the  opener  of  the  rima  glottidis.  (See 
Fig.  25,  C.)  From  this  "posticus  paralysis,"  when  it  is  bilateral,  there  results 
an  interference  with  respiration,  while  phonation  is  normal. 

11.  The  Spinal  Accessor;/  Nerve.  This  nerve  alone  supplies  the  sterno- 
cleidomastoid muscle,  while  in  the  innervation  of  the  trapezius  it  is  assisted  by 


•The  researches  of  J.  /.'.  Hunt  seem  to  show  thai  the  facial  nerve  has  also  a  sensory 
nini  which  supplies  a  cone-shaped  area  including  the  external  auditory  canal  and  a  mal] 
portl >f  the  outer  ear. — Translator. 


38  LECTURE    II 

the  upper  cervical  nerves.  If  the  spinal  accessory  is  destroyed,  there  is  a  com- 
plete paralysis  of  the  sternocleidomastoid.  Hence,  in  one-sided  lesion,  it  is 
impossible  to  turn  the  chin  completely  toward  the  opposite  side,  while  in  bi- 
lateral paralysis  there  is  also  a  tendency  for  the  head  to  fall  backward.  On 
the  other  hand,  the  paralysis  of  the  trapezius  is  incomplete  and  makes  itself 
evident  only  in  impaired  force  and  extent  of  the  elevation  of  the  arm. 

12.  The  Hypoglossal  Xerve. — The  hypoglossus  can  be  described  briefly 
as  the  nerve  supplying  the  tongue  muscles.  Indeed,  its  part  in  the  innervation 
of  the  lower  muscles  attached  to  the  hyoid  bone  (sternohyoid,  sternothyroid, 
omohyoid),  which  serve  in  part  to  fix  the  larynx,  is  only  apparent.  In  the 
so-called  "ansa  hypoglossi,"  through  which  this  innervation  occurs,  there  run 
fibers    from    the    cervical    nerves,   which    only    enter    the  hypoglossus    through 


Image  of  the  Vocal  Cords  on  Inspiration. 

A.  Normal. 

B.  Left-sided  vagus  paralysis  (Kecurrens  paralysis). 

C.  Bilateral    posterior   arytenoid    paralysis. 

anastomoses.  Bilateral  paralysis  of  the  hypoglossus,  of  course,  produces  a 
total  "glossoplegia" ;  following  the  influence  of  gravity  the  tongue  lies  im- 
movable upon  the  floor  of  the  mouth;  speech  is  unintelligible,  eating  is  consid- 
erably interfered  with.  In  unilateral  paralysis  (hemiglossoplegia),  on  the 
other  hand,  the  motor  disturbance  is  relatively  small;  indeed,  talking  and  eat- 
ing are  usually  not  at  all,  or  scarcely,  disturbed.  This  is  on  account  of  the 
multiple  interlacements  between  the  muscle  fibers  of  both  sides  of  the  tongue. 
If,  on  the  contrary,  the  patient  is  asked  to  show  his  tongue,  a  deviation  of  the 
tip  of  the  tongue  toward  the  side  of  the  paralysis  occurs,  on  account  of  the 
preponderance  of  action  of  the  genioglossus  on  the  sound  side. 

The  Occurrence  of  Peripheral  Interruptions  of  Conduction 
in  the  Cranial  Nerves 

Although  in  Lecture  I  (in  speaking  of  the  etiology  of  disturbances  of  con- 
duction in  peripheral  nerves)  we  had  the  cranial  nerves  also  in  mind,  and  for 
example,  considered  rheumatic  facial  paralysis,  I  will,  in  this  connection,  bring 
before  you  the  conditions  under  which  paralyses  of  a  peripheral  nature  of  indi- 
vidual cranial  nerves  come  under  observation.  According  to  the  localization  of 
interruption  of  conduction  of  this  nature,  in  a  given  cranial  nerve,  we  must  take 
into  consideration  certain  etiological  factors. 

The  olfactory  nerve  is  involved  most  frequently  in  injuries  to  the  head  with 


DISEASES    OF    THE    PERIPHERAL    NERVES  39 

or  without  fractures  of  the  skull,  but  also  in  tumors,  abscess  and  meningitis  in 
the  anterior  fo.ssa  T)f  the  skull. 

The  optic  nerve  can  be  affected  in  syphilis,  acute  infectious  diseases,  poison- 
ing (e.g.,  with  lead,  arsenic,  nicotin,  santonin,  iodoform,  quinine,  alcohol),  in 
severe  anemias  {e.g.,  after  hemorrhages  from  the  stomach  or  metrorrhagia),  in 
auto-intoxications  (diabetes,  nephritis),  also  by  neoplasms,  whether  these  arise 
from  the  nerve  itself  or  from  neighboring  structures  {e.g.,  from  the  orbit)  ; 
finally,  it  is  not  seldom  wounded  in  attempts  at  suicide  (by  a  shot  in  the  temple). 

Etiological  factors  for  disease  in  the  course  of  the  eye-muscle  nerves  are: 
fractures  at  the  base  of  the  skull  (the  abducens  is  especially  endangered  on  ac- 
count of  its  exposed  position  at  the  point  of  the  pyramid  of  the  petrous  bone)  ; 
compression  by  aneurisms  of  the  internal  carotid  or  by  tumors ;  otitis  media 
(abducens  paralysis);  acute  infectious  diseases;  diabetes,  syphilis,  nephritis, 
gout,  alcoholism,  chronic  lead  poisoning. 

Peripheral  interruptions  of  conduction  in  the  trigeminus  rarely  affect  sin- 
gle branches  only;  apart  from  injuries  of  the  bones  and  the  soft  parts  of  the 
face,  in  the  main  only  tumors,  tuberculous  and  syphilitic  processes  of  the  bones 
which  contain  the  foramina  of  exit  of  the  nerves,  and  of  their  periosteum  come 
into  consideration  as  causes.  The  Gasserian  ganglion  and  the  common  trunk 
of  the  nerve  are,  however,  much  more  frequently  affected  and,  indeed,  almost 
always  unilaterally  (aneurisms  of  the  internal  carotid,  tumors  of  the  internal 
carotid,  transverse  basal  fractures  behind  the  sella  turcica,  hypophysis  tumors, 
etc.). 

The  facial,  as  said  before,  is  by  far  most  frequently  affected  by  "rheu- 
matic" influences;  that  is,  without  any  other  recognizable  etiology  than  ex- 
posure to  cold.  In  "idiopathic  facial  paralysis"  even  this  etiological  factor  is 
sometimes  not  to  be  made  out.  Other  causes  are:  otitis  media,  meningitis, 
tumors  in  the  posterior  fossa  of  the  skull,  diseases  of  the  petrous  bone,  syphilis, 
erysipelas,  diphtheria,  protective  inoculation  against,  rabies.  Finally,  are  to  be 
mentioned  traumata,  which  are  quite  common  in  the  course  of  the  facial  nerve 
( fall  against  a  window-pane,  sabre  cuts  in  duels). 

The  auditory  nerve  is  affected  by  tumors,  diseases  of  the  meninges  and 
C&ries  of  bone  in  the  posterior  fossa  of  the  skull,  as  well  as  by  inflammations 
of  the  middle  car,  corresponding  to  its  close  relations  with  the  facial  nerve, 
quite  as  frequently  as  this  latter.  Also  fractures  of  the  base  of  the  skull  some- 
times involve  it.  Besides  different  acute  and  chronic  infectious  diseases  (typhoid 
fiver,  diphtheria,  influenza,  scarlel  fever,  parotitis,  tuberculosis,  syphilis),  leu- 
cemia  lias  been  specially  mentioned  as  an  etiological  factor  in  auditory  neuritis. 

Isolated  paralysis  of  the  glossopharyngeal  nerve  scarcely  ever  occurs.  Other 
nerves  are  usually  involved  at  the  same  time,  particularly  the  vagus,  especially 
where  the  interruption  of  conduction  is  of  traumatic  origin  (fractures  of  the 
base,  tumors,  the  pressure  of  aneurisms,  or  sinus  thromboses,  etc.).  The  vagus 
can  be  a  Heeled  Iii  many  kinds  of  poisonings  {e.g.,  carbonic  oxide,  morphine, 
atropin,  phosphorus,  lead,  arsenic,  alcohol),  as  well  as  in  connection  with  dif- 
ferent acute  infectious  diseases.  Paralysis  of  the  recurrent  laryngeal  nerve 
is,  as  a  rule,  caused  by  local  anomalies  in  the  neck  or  in  the  mediastinum  (si  ruina, 
tumors,  enlarged  glands,  etc.). 


40  LECTURE    II 

The  spinal  accessory  and  hypoglossals  are  particularly  exposed  to  trau- 
matic injury  and  upon  the  basis  of  the  same  causal  factors  as  the  glossopharyn- 
geal and  vagus.  Hypoglossal  neuritis  occurs  also  in  intoxications  (alcohol, 
lead,  arsenic,  carbonic  oxide). 


B.     Special  Remarks  on  Neuritis  and  Polyneuritis 

I.     IRRITATIVE   SYMPTOMS 

While  we  might  consider  disturbances  of  conduction,  whose  general  and  spe- 
cial phenomenology  we  have  already  learned,  as  a  general  characteristic,  as  well 
of  traumatic  and  neoplastic,  as  of  neuritic  diseases  of  the  peripheral  nerves, 
we  must  now  enumerate  the  irritative  symptoms,  which  play  a  not  less  important 
role  in  the  clinical  picture  of  the  last-mentioned  nosological  group. 

They  manifest  themselves  to  by  far  the  greatest  extent  as  disturbances 
of  sensation:  that  is,  as  pain  along  the  course  of  the  affected  nerves.  They  may 
have  a  shooting,  stabbing,  or  boring  character,  are  sometimes  described  as  a 
burning  on  the  surface  of  the  skin  (the  "causalgia"  of  Weir-Mitchell).  From 
these  pains  proper,  they  pass  by  imperceptible  degrees  to  the  "paresthesias," 
the  more  or  less  painful  abnormal  sensations  which  are  often  felt  in  the  terri- 
tory of  the  affected  nerves:  formication,  prickling,  tickling,  subjective  feeling 
of  cold  or  heat,  etc.  Also  hyperesthesia  to  touch,  on  account  of  which  the  pres- 
sure of  the  clothing  or  of  the  bedclothes  may  be  unbearable,  is  not  infrequent. 
Here  and  there,  also,  spontaneous  pains  are  projected  into  skin  regions  in 
which,  by  objective  tests,  touch  and  pain  sense  have  been  proved  to  be  reduced 
(hypesthesia  dolorosa,  hypalgesia  dolorosa).  Further,  it  sometimes  occurs 
that  pains,  at  least  for  a  time,  are  located  in  the  joints,  and  so  arthritic  dis- 
eases are  simulated.  The  irritation  of  fibers  conducting  centripctally  can  ex- 
ceptionally be  accompanied  by  exaggeration  of  reflexes.  The  conditions  under 
which  the  influence  of  the  disease  in  exaggerating  the  reflexes,  overcomes  the 
effect  of  lesions  of  the  afferent  or  efferent  part  of  the  reflex  arc  in  destroying 
the  reflexes,  are  not  clear  to  us.  Perhaps  in  such  cases  the  perineuritic  processes 
predominate  anatomically  over  the  parenchymatous,  since  the  spontaneous 
pains  may  well  depend  upon  perineuritic  changes.  That  in  general,  the  irrita- 
tive phenomena  in  the  centripetal  fibers  (pain,  hyperreflexia)  occur  most  usu- 
ally in  acute  neuritides  and  polyneuritides,  agrees  with  what  was  a  priori  to  be 
expected. 

Motor  irritative  phenomena  are,  on  the  other  hand,  very  infrequent,  and 
have  been  described  almost  exclusively  in  multiple  neuritis,  e.g.,  cramps  in  the 
calves  in  localization  of  the  disease  in  the  legs.  Babinski  asserts  that  there 
is  often  a  latent  predisposition  to  cramps  in  the  calves  which  can  be  provoked 
by  faradization ;  I  have  so  far  not  been  able  to  convince  myself  of  this.  On  the 
other  hand,  I  saw  a  peculiar  muscle  wave  ("myokymia"),  which  has  also  been 
corroborated  by  Remdk,  once  in  weak  development  in  the  peroneal  muscles  in 
a  tuberculous  polyneuritis,  very  plainly  in  the  biceps  brachii  in  an  alcoholic 
neuritis. 


DISEASES    OF    THE    PERIPHERAL    NERVES  41 


II.     PALPATORY    FINDINGS 

Abnormal  findings  by  palpation  of  superficially  situated  nerves  are  quite 
frequent.  In  the  first  place,  there  is  more  or  less  marked  sensitiveness,  so  that 
pain  is  produced  by  moderate  pressure — upon  the  brachial  plexus  above  the 
clavicle  or  in  the  axilla,  upon  the  nerve  structures  in  the  internal  bicipital  sulcus, 
upon  the  ulnar  on  the  epicondyle,  upon  the  musculo-spiral  in  the  groove  on 
the  outside  of  the  humerus,  upon  the  peroneal  behind  the  head  of  the  fibula, 
upon  the  sciatic  between  the  flexors  of  the  knee,  etc.  A  certain  familiarity 
with  the  intensity  of  the  pressure  which  normally  is  necessary  to  produce  pain 
in  these  different  places  is  needed  in  testing  for  this  phenomenon ;  for  example, 
when  it  is  necessary  to  elicit  it  by  comparison  with  the  healthy  corresponding 
nerve  on  the  opposite  side  (in  polyneuritis).  Less  frequent  than  abnormal  sen- 
sitiveness to  pressure,  but  by  no  means  infrequent,  a  thickening  of  the  nerve 
trunks  can  sometimes  be  felt  upon  palpation.  This  can  well  be  the  expression 
of  a  perineuritic  process ;  such  a  process  appears  to  be  the  cause  of  the  sensi- 
tiveness to  pressure  of  nerves  afflicted  with  neuritis,  which  can  occur  in  purely 
motor  branches  and  is  in  this  case  attributable  to  the  irritation  of  the  "nervi 
nervorum."  In  order  to  determine  by  palpation  that  there  is  a  definite  swelling 
of  a  nerve,  industrious  practice  in  palpation  of  normal  peripheral  nerve  trunks 
is  even  more  necessary  than  in  the  testing  of  sensitiveness  to  pressure.  It  is 
well,  also,  not  to  lose  any  opportunity  which  is  presented  to  control  one's  pal- 
patory findings  anatomically.  For  example,  I  have  found  on  autopsy,  or  on 
operative  exposure,  nerves  which  appeared  swollen  by  palpation  actually  two 
or  three  times  as  thick  as  the  corresponding  portions  of  normal  nerves. 

III.     THE    DIFFERENT    CLINICAL   FORMS 

The  neuritides  may  be  grouped  according  to  different  principles ;  for  exam- 
ple: 1,  according  to  their  course  into  acute,  subchronic  and  chronic;  2,  accord- 
ing to  the  nature  of  their  symptoms  into  motor,  "mixed"  and  sensory;  .'5.  ac- 
cording to  their  extent,  into  mononeuritides,  disseminated  neuritides,  plexus- 
neuritides  and  po] yneuritidcs.* 

The  acute  neuritic  diseases  belong  for  the  greater  part  to  the  etiological 

*  Of  late  attention  has  been  directed  to  the  "root-neuritides"  ("radiculitides").  These 
conditions  differ  anatomically  from  the  ordinary  neuritides  by  being  located  proximalrj  to  the 
plexuses  for  the  extremities— that  is,  in  the  region  of  the  spinal  nerve  routs;  clinically,  by 
the  t'.iet  thaPthe  pains  arising  in  consequence  of  them  an-  located,  not  in  the  territories  of 
the  peripheral  nerves,  hot  in  the  spinal  segmentary  areas  of  innervation.  (See,  farther  cm. 
Lecture  XI.)  Etiologically,  infections  and  intoxications  play  the  chief  role,  as  in  ordinary 
neuritides.  Ordinarily  the  disease  manifests  itself  in  two  phases:  :[  stage  of  irritation  with 
attacks  of  pain,  hyperesthesia  of  the  shin,  and  sometimes  exaggeration  of  reflexes,  and  a  stage 
of  destruction  with  anesthesia,  paralysis  and  absence  of  reflexes.  The  pains  have  the  "lanci- 
nating" and  specially  severe  character  typical  for  radicular  affections  {e.g.,  for  tubes  or 
carcinoma  of  the  vertebra; — sec  below).  They  are  often  sel  up  bj  movements  or  by  over- 
exertion— in  specially  characteristic  manner  by  sneezing.     Sensitiveness  to  pressure,  or  u| 

palpation   of  accessible   peripheral   nerves,   i.s   not    present    or   scarcely    perceptible.     Painful 
points  nvcr  the  spinous  processes  are  recognizable,  however, 


42 


LECTURE    II 


group  of  infectious  and  rheumatic  neuritides ;  they  also  arise,  however,  upon 
the  basis  of  intoxications ;  for  example,  in  lead,  arsenic,  and  carbonic  oxide 
poisoning.  In  my  first  lecture  I  mentioned  a  case  due  to  the  combined  action 
of  bromide  of  methyl  and  methyl  alcohol,  which  had  an  acute  course.  The 
so-called  "acute  curable  ataxia"  of  drinkers  is  a  by  no  means  seldom  form  of 
alcoholic  polyneuritis ;  an  "apoplectiform  alcoholic  paralysis"  was  first  shown 
on  autopsy,  by  Eichhorst,  to  be  a  polyneuritis  beginning  hyperacutely.  Cer- 
tain polyneuritides  which  ascend  from  the  lower  extremities  in  "foudroyant" 
manner  and  rapidly  cause  death  by  paralysis  of  the  heart  and  respiratory  nerves 
are  denominated  the  "neuritic  form  of  Landry's  paralysis";  if  an  infectious 
basis  is  here  the  rule,  this  unfavorable  course  can  also  be  a  manifestation  of 
alcoholic  polyneuritis.  Chronicity  distinguishes  in  general  the  arterio-sclerotic, 
senile,  anemic,  cachectic,  diabetic,  and  also  the  majority  of  alcoholic  and  tuber- 
culous neuritides  and  polyneuritides. 

In  carrying  out  the  division  into  motor,  "mixed,"  and  sensory  neuritides, 
it  is  at  once  apparent  how  infrequent  the  last  are;  apart  from  the  neuritis  of 


Fig.  26. 
Tuberculous   Polyneuritis.      General   Muscular   Atrophy. 


the  external  cutaneous  nerve  of  the  thigh,  the  so-called  "paresthetic  meral- 
gia,"  we  find  in  the  literature  very  few  descriptions  of  sensory  neuritides 
{e.g.,  isolated  neuritis  of  the  lateral  cutaneous  peroneal,  of  the  internal  cutane- 
ous of  the  thigh,  of  the  chief  sensory  branches  of  the  radial),  and  also  those 
polyneuritides  in  which  every  electrodiagnostic  and  dynamometric  sign  of  an  in- 
volvement of  the  motor  fibers  is  absent,  form  a  quite  small  minority.  Pure 
motor  neuritides  are,  on  the  other  hand,  more  frequent  and  most  remarkable, 
even  when  the  affected  nerves  contain  sensory  fibers.  A  certain  "electivity" 
in  the  action  of  the  disease-producing  cause,  which  we  will  consider  more  fully 
farther  on  in  this  lecture,  is  evident  in  these  cases.  Nevertheless,  I  have  found, 
that  by  the  use  of  finer  methods  of  testing  sensibility  {e.g.,  by  the  tuning- 
fork),  a  large  number  of  mononeuritides  and  polyneuritides,  which  at  first 
sight  appear  to  be  purely  motor,  later  turn  out  to  be  of  the  "mixed"  form. 

The  above  mentioned  "meralgia  paraesthetica"  is  a  not  so  rare  clinical 
picture  described  by  Rotli  and  Bernhardt.  The  peculiar  relations  of  the  external 
cutaneous  nerve  in  its  passage  through  the  fascia  of  the  thigh,  appear  to  pre- 
dispose this  nerve  to  destructive  lesions.  As  exciting  causes,  infectious  diseases, 
overexertion,    abnormal    static    relations    (flat    foot)    diabetes,    arteriosclerosis 


DISEASES    OF    THE    PERIPHERAL    NERVES  43 

and  nicotinism,  have  been  mentioned.  The  disease  is  usually  unilateral.  The 
patients  complain  of  the  most  varied  paresthesias  and  spontaneous  pains  in 
the  region  of  distribution  of  the  external  cutaneous  nerve;  often  certain  points 
are  also  very  sensitive  to  pressure.  The  hyperesthesia  of  the  skin  can  cause 
the  friction  of  the  clothing  to  be  painful.  Objectively  hypesthesia  or  anesthe- 
sia in  a  more  or  less  extensive  portion  of  the  territory  in  question  is  found. 
Meralgia  is  in  general  persistent  and  shows  a  tendency  to  relapses. 

We  speak  of  mononeuritis  when  the  territory  of  only  one  nerve  is  affected, 
of  total  or  partial  plexus  neuritis  when  a  nerve  plexus,  for  example  the  brachial 
plexus,  is  attacked  in  its  entirety  or  witli  the  escape  of  single  branches.  In  a 
disseminated  neuritis  single  nerves  are  attacked  at  the  same  time  without  any 
regularity  in  the  distribution  and  development  of  the  paralysis.  In  contradis- 
tinction to  this,  we  reserve  the  term  polyneuritis  for  cases  of  more  or  less 
diffuse  disease  of  the  peripheral  nervous  system,  which  present  a  nearly  sym- 
metrical distribution  and  run  a  regular  course.  We  will  now  devote  some  space 
to  these  forms. 

According  to  their  extent,  poh-neuritides  may  be  divided  into  those  of 
paraplegic  or  tetraplegic  type,  those  which  run  their  course  as  symmetric  arm 
plexus  neuritis  without  involvement  of  the  legs,  and  finally  those  in  which  the 
cranial  nerves  are  exclusively  attacked  or  are  affected  along  with  the  others. 
It  is  noteworthy  that  according  to  the  special  etiology,  different  topographical 
and  physiopathological  peculiarities  are  regularly  present.  Lead  neuritis 
avoids  the  sensory  element  and  affects  (where  by  overexertion  of  special  groups 
of  muscles  the  localization  is  not  influenced  in  the  sense  of  the  "toxico-pro- 
fessional"  paralysis  of  Oppenheim  (sec  above),  particularly  the  muscles  of 
the  forearm  supplied  by  the  radial  nerve;  very  rarely,  generalized  lead  paral- 
yses, in  which  loss  of  motor  power  affects  the  territory  of  one  nerve  after 
another  symmetrically,  either  in  exacerbations,  or  in  rapid  succession,  some- 
times under  febrile  movement,  occur.  In  these,  however,  the  predisposed  muscle 
groups  are  more  seriously  affected  than  the  others.  Arsenical  polyneuritis, 
which  usually  produces  both  motor  and  sensory  symptoms,  as  far  as  its  motor 
components  are  concerned,  presents  in  so  far  the  contrary  of  saturnine 
polyneuritis  in  th.it  the  lower  extremities  are  attacked  by  preference.  Among 
72  cases  of  Brouardel's  they  were  affected  first  in  69  cases,  exclusively  in  .'55 
cases.  However,  when  the  anus  are  affected,  the  atrophic  paralysis  of  the 
ulnar  and  median  nerves  by  far  predominates,  while  the  radial  paralysis  typi- 
cal of  lead  intoxication  occupies  the  background.  Characteristic  of  diphtheritic 
polyneuritis  is,  on  the  one  hand  its  preference  for  certain  centripetal  nerve 
fillers  from  the  lower  limbs,  which  lead  to  a  "post  diphtheritic  ataxia";  on  the 
other  a  decided  affinity  for  motor  nerves  from  the  proximal  parts  of  the  cerebro- 
spinal apparatus.  Wwr  the  paralysis  of  the  palate  and  the  muscles  of  accom- 
modation of  the  eve  (  the  ciliary)  by  far  predominate.  Fortunately  the  pharyn- 
geal, diaphragm,  and  heart  paralyses,  so  dangerous  to  life,  are  rare;  the  exten 
sion  of  the  polyneuritic  process  to  the  innervation  of  the  neck  and  external 
eye  muscles  is  also  rare.  I  only  once  observed  a  symmetrica]  accessorius  paral- 
ysis. However,  diphtheritic  paralyses  of  tin-  muscles  of  the  extremities,  which 
arc    guite    inconstant    in    their    topographical    distribution,    also    occur.      The 


44  LECTURE    II 

polyneuritides  from  the  inhalation  of  sulphide  of  carbon  affect  mainly  the 
flexors  of  the  hand  and  the  extensors  of  the  foot.  These  few  examples  may 
serve  as  instances  of  the  characteristic  choice  which  certain  nerve  poisons  exer- 
cise, with  noteworthy  regularity,  in  the  production  of  polyneuritic  symptom- 
complexes. 

This  "electivity"  speaks  in  the  sense  of  Ehrlich's  doctrine  of  "organotropy," 
for  the  idea  that  certain  poisons  possess  certain  chemical  affinities  for  certain 
cell  substances.  According  to  the  chemotherapeutic  investigations  of  Ehrlich 
the  different  arsenic  preparations,  for  example,  may  be  arranged  in  an  almost 
continuous  scale  as  regards  their  "neurotropy."  In  spite  of  this,  in  the  assump- 
tion of  an  elective  chemical  action  of  the  poisons  causing  neuritis,  we  come 
up  against  the  fact  that  clinically  as  well  as  anatomically,  the  symptoms  mani- 
fest themselves  preponderantly  or  exclusively  in  the  periphery  of  the  neurones 
in  question.  A  second  factor  comes  here  into  the  question:  the  greater  the 
distance  from  the  trophic  centre  (that  is,  from  the  spinal  cord  and  the  spinal 
ganglia)  the  less  the  power  of  resistance  of  the  neurone  to  the  action  of  toxic 
substances.  Here  the  "Ersatz"  theory  of  Edinger,  already  mentioned  in  Lec- 
ture I,  can  with  advantage  be  taken  into  consideration. 


LECTURE  III 
Diseases  of  the  Peripheral  Nerves 

C.     The  Neuralgias 

Pains  which  occur  in  attacks  and  radiate  throughout  the  territory  of  dis- 
tribution of  peripheral  nerves  we  call  neuralgia.*  As  to  the  material  basis  of 
this  characteristic  symptom,  we  are  very  poorly  informed ;  however,  in  the 
vast  majority  of  cases  in  which  the  nerves  attacked  have  been  examined  mi- 
croscopically, they  have  been  found  entirely  normal.  Oppcnhcim  suggests 
with  justice,  that  fine  disturbances  of  nutrition  of  the  nerve  (perhaps  also 
of  its  nervi  nervorum)  lie  at  the  base  of  the  neuralgias,  disturbances  which 
only  exceptionally  lead  to  visible  alterations,  which  then  approach  the  anatom- 
ical picture  of  neuritis.  The  cause  of  these  disturbances  of  nutrition  is  only 
in  a  minority  of  the  cases  of  mechanical  nature;  for  example,  a  stasis  of  the 
veins  accompanying  the  nerve,  through  which  the  latter  is  compressed  in  a 
bony  canal  or  in  some  other  channel  (Bardenheuer) ,  as  perineuritic  adhesions, 
osteitis,  periostitis,  tumors,  cicatrices,  etc.,  which  exert  a  traction  or  com- 
pression upon  the  nerve,  or,  finally,  a  trauma  ;  usually,  however,  general  toxemic 
influences  are  to  be  held  responsible.  Of  the  infectious  diseases,  influenza, 
malaria  and  syphilis  play  the  greatest  role  in  the  etiology  of  the  neuralgias; 
of  diseases  of  metabolism,  diabetes  and  gout,  of  other  auto-intoxications, 
chronic  constipation,  of  exogenic  poisonings,  alcoholism.  Undoubtedly  the 
"nervous"  (viz.,  neurasthenics  and  hysterics),  are  just  as  much  predisposed 
to  neuralgias  as  anemic  and  chlorotic  individuals.  Further,  the  influence  of 
refrigeration,   especially  of  circumscribed   nature,   as,   for  example,   a   draught 

01 e  side  in  trigeminus  neuralgia,  sitting  on  cold  stones  in  sciatica,  is  not 

to  be  denied.  I  have  already  indicated  what  can  be  said  about  the  rheumatic 
origin  of  diseases  of  the  peripheral  nerves. 

Finally,  the  so-called  "reflex"  neuralgias  are  to  be  considered;  problematic 
is  this  method  of  origin  for  such  cases  in  which  facial  neuralgias  have  been 
brought  into  causal  connection  with  a  retroflexion  of  the  uterus.  Better  founded, 
on  the  contrary,  are  the  very  frequent  connection  of  trigeminal  neuralgia  with 
Uncorrected  anomalies  of  refraction  of  the  eve,  caries  of  the  teeth,  suppuration 

of  the  middle  ear,  catarrhs  of  the  frontal  sinus  and  of  the  ant  ruin  of  High/more, 
that   of  sciatica  with  flat   foot,  etc.      Puberty   and   the   period   of  retrogres 

*  There  are,  indeed  (.-is  complement  to  the  root  neuritides  described  in  the  footnote  on 
page  II),  radicular  neuralgias  (root  neuralgias,  radiculalgias)  in  which  the  pain  radiates, 
not  in  the  anatomical  distribution  01  the  peripheral  nerves,  I  <  1 1 1  presents  the  character  of  the 
lancinating  pains  of  tabes  dorsalis,  to  be  described  in  Lecture  XI, 

45 


46  LECTURE    III 

sive  change,  seem  the  times  of  special  predisposition  for  neuralgias.  Men  are 
affected  much  more  frequently  than  women ;  nevertheless,  during  pregnancy 
and  in  the  puerperium,  the  latter  are  decidedly  more  susceptible. 

THE  GENERAL  SYMPTOMATOLOGY  OF  NEURALGIA 

The  attack  of  neuralgic  pain  either  appears  with  lightning-like  suddenness, 
or  unpleasant  sensations  (a  feeling  of  heat,  of  tension,  of  formication,  etc.),  in 
the  distribution  of  the  nerve  affected,  precede  it  by  some  time.  Between  the  single 
attacks  there  is  either  entire  freedom  from  pain,  a  dull  pain  of  little  intensity, 
or  a  feeling  of  irritation  and  soreness  of  the  neuralgic  nerve  is  complained  of. 
Sometimes  definite  exciting  causes  can  be  held  responsible  for  the  outbreak  of 
the  attack  of  pain;  for  example,  sudden  movements,  a  cold  draught,  meteorolog- 
ical influences,  mental  excitement;  often,  however,  we  seek  in  vain  for  such 
exciting  factors. 

The  pain  has  usually  a  tearing,  dragging  character,  sometimes  it  is  de- 
scribed as  burning,  cutting  or  boring;  it  is  usually  not  continuous,  but  con- 
sists of  paroxysms  rapidly  following  one  another ;  frequently  these  are  so  in- 
tense that  the  patients  grow  pale,  break  out  in  a  sweat,  groan,  or  even  scream. 
The  duration  of  the  attack  is  varied,  sometimes  very  short  (a  quarter  or  half 
minute),  mostly  a  few  minutes:  sometimes,  however,  lasting  for  hours.  Char- 
acteristic of  neuralgia  is  the  fact  that  the  pain  is  always  quite  sharply  lo- 
calized by  the  patient  so  that  he  sometimes  can  demonstrate  to  us  the  whole 
course  of  the  painful  nerve  with  anatomical  correctness ;  in  severe  cases,  indeed, 
the  irradiation  of  the  pain  into  a  neighboring  nerve  territory  is  a  frequent 
occurrence  at  the  height  of  the  attack.  Objective  accompanying  symptoms 
are  only  to  be  made  out  in  part  of  the  cases ;  most  frequent  appears  to  me  to 
be  the  reddening  of  the  integument  in  the  region  of  the  affected  nerve ;  this  can, 
indeed,  outlast  the  attack  and  can  be  observed  between  the  attacks.  The  same 
remarks  apply  to  the  much  more  infrequent  finding  of  a  slight  oedema  of  the 
skin.  I  would  mention,  further,  the  motor  symptoms  accompanying  severe  at- 
tacks of  neuralgia:  a  tension  of  the  muscles  of  the  region  during  the  attack, 
sometimes,  also,  clonic  contractions.  In  neuralgias  of  the  extremities  of  long 
duration,  in  consequence  of  the  position  which  the  patient  causes  the  affected 
limb  to  assume  in  his  effort  to  avoid  the  pain,  there  is  often  a  decided  degree 
of  emaciation. 

A  hyperesthesia  of  the  skin  in  the  affected  territory  can  quite  frequently  be 
made  out  and,  on  account  of  this,  pressure  and  the  friction  of  the  clothing  often 
become  unbearable.  Less  frequently  there  is  slight  hypoesthesia.  More  im- 
portant for  diagnosis  is  the  sensitiveness  to  pressure  of  the  affected  nerve, 
which  is  mainly  limited  to  definite  points  in  its  course  (where  it  leaves  a  bony 
canal,  perforates  a  fascia  lies  upon  a  firm  tissue)  ;  exceptionally,  however,  af- 
fects the  whole  of  its  course  accessible  to  palpation.  These  so-called  "Valleix 
points"  can,  as  a  rule,  also  be  demonstrated  independent  of  the  attacks  of  pain; 
pressure  exerted  upon  them  can,  indeed,  provoke  the  attack,  while,  on  the 
other  hand,  in  many  cases  firm  pressure  upon  these  points  during  the  attack 
is  perceived  as  ameliorative.     In  describing  the  special  forms  of  neuralgia  we 


DISEASES    OF    THE    PERIPHERAL    NERVES  47 

will  further  consider  the  Valleix  points,  which,  however,  are  no  necessary  ac- 
companiment of  neuralgias.* 


THE    MOST    IMPORTANT    FORMS    OF    NEURALGIA 

A.      Trigeminus   Neuralgia 

Among  the  general  causative  factors  of  this  very  frequent  form  of  neuralgia, 
for  which,  also,  the  names  of  prosopalgia  and  "FothergUTs  face  pain"  are  occa- 
sionally' used,  diseases  of  metabolism  and  infectious  diseases  play  a  great  role. 
Among  these  there  is  in  so  far  a  certain  electivity  in  that,  for  example,  malarial 
and  influenzal  neuralgias  affect  the  first  branch,  diabetes  and  syphilis  the  third 
branch,  by  preference.  This  last  in  contradistinction  to  the  first  branch  which 
is  almost  without  exception  affected  on  one  side  alone,  can  be  bilaterally 
involved. 

(Specially  characteristic  of  syphilis  is  the  bilateral  Seeligmueller's  neural- 
gia of  the  auriculotemporal  nerve,  in  which  the  pain  "like  a  child's  comb"  runs 
over  the  vertex  from  one  ear  to  the  other.)  Among  local  causes,  caries  of  the 
teeth,  pyorrhoea  alveolaris,  osteitis  alveolaris  in  people  without  teeth  ("nev- 
ralgie  des  edentes"),  empyema  of  the  antrum  of  Highmore,  or  of  the  frontal 
sinus,  diseases  of  the  nose,  ears  and  eyes,  must  particularly  be  considered  and  in 
obscure  cases  there  should  be  no  hesitation  in  enlisting  the  services  of  the  dentist; 
the  oculist  or  the  oto-rhinologist. 

Not  infrequently  the  attack  of  pain  comes  on  at  some  definite  time  of  day. 
for  instance,  on  awakening  in  the  morning,  or  at  night  (nocturnal  neuralgia 
of  Oppenheim).  Chewing,  yawning,  sneezing,  pressing  the  teeth  together,  blow- 
ing the  nose,  speaking,  often  set  up  the  attack,  so  that  many  of  these  patients 
live  in  continual  anxiety  with  regard  to  these  "provocative  agents,"  withdraw 
from  all  society,  nourish  themselves  only  insufficiently  with  liquid  food,  etc. 
Sometimes  they  discover  for  themselves  certain  procedures  which  can  cut  short 
the  attack;  for  example,  in  neuralgia  of  the  mandibular  branch,  the  production 
of  negative  pressure  in  the  mouth  by  movements  of  sucking,  the  mouth  being 
closed,  in  that  of  the  ophthalmic  branch  by  energetic  pressure  upon  a  Valleix's 
point.  The  whole  trigeminus  is  seldom  affected,  by  far  most  frequently  the 
ophthalmic  branch,  particularly  its  division  supplying  the  forehead  ("supra 
Orbital  neuralgia");  there  is  also  a  "ciliary  neuralgia"  which  has  its  location 
behind  the  eyeball.  Neuralgia  of  the  maxillary  branch  affects  by  preference 
the  infra-orbital  nerve;  it  is  somewhat  more  frequent  than  that  of  the  man- 
dibular. 

On  page  +8  the  Valleix  pressure  points  in  trigeminus  neuralgia  are  exposed 
in   tabular   form. 


'  II  i-.  somewhat  impressive  thai  pressure  upon  Valleix's  points  causes  local  pain  instead 
nf  pain  projected  into  !!»•  terminal  branches  of  tin-  nerve.  We  know,  for  example,  thai 
irritation  sei  up  in  an  amputation  stump  is  often  perceived  as  pain  in  Angers  or  lues.  Prob- 
»blj  the  sensitiveness  nf  tin-  pressure  points  depends  upon  .'in  irrilati f  the  nervi  nervorum. 


48 


LECTURE    III 


I  Branch  II  Branch                III  Branch 

1.  "Supraorbital    point,"    over  1.  "Infraorbital  point,"  at  the  1.  "Mental    point,"    over    the 

the     supraorbital     fora-  exit    of   the    infraorbital              mental   foramen 

men  nerve  in  the  canine  fossa 


2.  "Nasal      point,"     somewhat 

inside  of  the  inner  can- 
thus 

3.  "Palpebral  point,"  laterally 

from  the  upper  eyelid 
(exit  of  the  lacrymal 
nerve) 


J.  "Malar  point."  at  the  exit 
of  the  malar  branch  on 
the  zygoma  (For.  zygo- 
matico-faciale) 

3.  "Dental  points"  on  the 
upper  gum 


"Auriculotemporal  point," 
in  front  of  the  ear  on 
the  zygomatic  process 

"Temporal  point" 


4.  "Parietal    point,"    both    in 

the  course  of  the  auri- 
culotemporal nerve,  be- 
tween the  auriculotem- 
poral point  and  the  ver- 
tex 

5.  "Dental     points,"     on     the 

lower  gum 


Of  the  occasional,  but  in  no  way  regular,  accompanying  symptoms  of  tri- 
geminal neuralgia,  the  sensible  and  sensory  disturbances  must  next  be  mentioned ; 
most  frequently  a  hyperesthesia  in  the  skin  territory  of  the  diseased  nerve  is 
to  be  found.  This  is  usually  particularly  marked,  on  touching  the  part  with 
a  cold  object  (a  piece  of  metal)  ;  many  patients  evince  great  pain  from  this  test. 
In  inveterate  neuralgias  we  find,  however,  here  and  there  a  slight  reduction  of 
cutaneous  sensibility,  while  cases  with  actual  anesthesia,  are  hardly  any  longer 
to  be  classed  as  trigeminal  neuralgia.  The  attacks  are  often  accompanied  by 
photophobia,  less  frequently  by  narrowing  of  the  visual  field,  difficulty  in  hearing 
or  disturbances  of  taste.  Vasomotor,  secretory  and  trophic  disturbances  have 
been  observed.  I  will  content  myself  with  mentioning  the  most  important  and 
interesting:  heat  and  redness  of  the  skin  of  the  face,  of  the  conjunctiva  and  of 
the  mucous  membrane  of  the  mouth  (upon  which,  indeed,  small  extravasations 
of  blood  may  occur),  increased  salivation,  lacrymation,  rhinorrhoea,  cedema, 
chemosis  of  the  conjunctiva,  the  eruption  of  herpes  vesicles;  in  inveterate  cases, 
circumscribed  falling  out  of  the  hair,  decolorization  of  the  hair,  and  atrophy  of 
the  integument.  Finally,  accompanying  motor  symptoms,  are  the  chronic  con- 
tractions of  the  muscles  of  the  face,  less  frequently  of  the  masticatory  muscles 
occurring  with  severe  attacks,  which  justify  the  denomination  "tic  douloureux" 
or  "spasmodic  epileptiform  neuralgia"  (Trousseau). 

The  prognosis  of  trigeminal  neuralgia  cannot  be  made  on  general  principles, 
but  only  through  intimate  acquaintance  with  the  clinical  peculiarities  of  the 
individual  case.  There  are  forms  which  are  cured  completely  and  finally  in  a 
few  weeks,  while,  on  the  other  hand  (particularly  in  old  age),  there  are  those 
which  are  refractory  to  every  kind  of  therapy  and  which  drive  the  patients  to 
despair,  so  that  suicides  have  been  frequently  observed.  Even  in  mild  cases 
the  tendency  to  recur  is  very  common,  so  that  the  history  of  the  disease  lasting 
years  and  decades  is  nothing  unusual. 

The  clinical  peculiarities  by  which  the  prognostically  more  favorable  cases 
are  distinguished  from  the  more  severe  ones  are  the  following: 

"Neuralgia  major"   has   the  most  marked  paroxysmal   character   in   that 


DISEASES    OF    THE    PERIPHERAL    NERVES  49 

the  pain  is  not  present  between  the  single  attacks ;  these,  however,  begin  with 
lightning-like  suddenness  and  usually  with  great  severity.  In  the  most  severe 
cases,  however,  they  follow  so  close  upon  one  another  that  they  make  the  impres- 
sion of  a  continued  pain.  Typical  is  the  provocation  of  these  attacks  by  move- 
ments, by  speaking,  by  chewing,  by  swallowing.  The  pressure  points  are  very 
often  not  at  the  points  of  exit  of  the  nerves,  but  upon  the  tooth-alveoli.  In 
general,  only  one  branch  is  affected,  usually  the  maxillary;  later,  however,  the 
ophthalmic  and  mandibular  may  be  involved.  Sometimes  in  the  course  of  the 
disease  there  occur  spasms  which  are  now  of  involuntary  character  ("tic 
douloureux"),  and  again  are  voluntary  (chewing  movements,  grimacing)  ; 
secretory  and  vasomotor  disturbances  occur. 

In  "neuralgia  minor,"  on  the  other  hand,  even  between  the  attacks  there 
is  a  certain  amount  of  pain,  from  which  the  attacks  distinguish  themselves 
as  exacerbations.  Valleix's  points  are  to  be  found  particularly  at  the  points 
of  exits  of  the  nerves,  even  between  the  paroxysms.  The  localizations  of  choice 
are  the  first  and  third  branches,  the  second  is  less  frequently  affected,  only  very 
exceptionally  the  trouble  involves  the  whole  of  the  trigeminus.  Motor,  secretory 
and  vasomotor  symptoms  are  absent.  Peripheral  etiological  factors  (affections 
of  the  nose,  the  eye,  the  teeth)  can  often  be  made  out. 

b.     Occipital  Neuralgia 

This  is  less  frequent  than  trigeminal  neuralgia  and  attacks  the  nervus  oc- 
cipitalis major,  oftener  than  the  nervus  occipitalis  minor.  Valleix's  points  are, 
for  the  first,  midway  between  the  mastoid  process  and  the  upper  cervical  verte- 
bra- on  the  linea  nucha5  superior  laterally  from  the  insertion  of  the  ligamentum 
nucha;  for  the  latter  nerve,  the  region  between  the  insertions  of  the  sternoclei- 
domastoid and  the  trapezius,  as  well  as  over  the  mastoid  process  itself.  The 
pains  radiate  over  the  occipital  region  to  the  vertex  and  are  often  bilateral. 
Etiologically,  besides  traumatic  influences  affecting  the  region  of  the  neck, 
gout,  malaria,  influenza  and  typhoid  fever  come  into  consideration. 

c.     Neuralgia  of  the  Phrenic  Nerve 

This  rare  neuralgia  depends  particularly  upon  local  causes,  pleurisy,  peri- 
carditis, aortic  lesions,  fractures  of  the  clavicle.  The  attack  of  pain  is  often 
initiated  by  coughing,  swallowing  or  drawing  a  deep  breath;  the  pain  radiates 
from  the  base  of  the  thorax  toward  the  neck,  sometimes  also  toward  the 
Bhoulder,  the  mastoid  process,  or  the  hand  (which  is  explained  by  the  anas- 
tomoses of  the  phrenic  with  the  other  branches  of  the  brachial  plexus).  The 
following  pressure  points  have  been  described:  I.  At  the  insertion  of  the 
diaphragm  into  the  tenth  rib,  somewhat  lateral  from  the  linea  alba  ("bouton 
diaphragmatic) lie"  of  the  French) .  '-'.  In  I  he  neck,  in  front  of  the  scalenus  ante 
rior.    3.  At  the  border  between  the  bone  and  cartilage  of  the  fifth  rib. 


50  LECTURE    III 

d.     Brachial,  Neuralgias 

Apart  from  ulnar  neuralgia  in  which  gout  is  always  to  be  considered  in 
the  etiology,  true  brachial  neuralgias  or  "brachialgias"  are  rare.  (On  this 
account  be  cautious  in  making  this  diagnosis.)  Neuralgiform  pains  in  the  arms 
should  always  arouse  a  suspicion  of  disease  of  the  vertebra3  or  the  meninges 
and  tumors  in  the  region  of  the  plexus.  Pressure  points  are,  for  the  musculo- 
spiral  the  place  where  it  runs  around  the  humerus  in  the  sulcus  spiralis ;  for  the 
ulnar,  back  of  the  internal  condyle ;  for  the  median,  the  bend  of  the  elbow  and 
the  palmar  surface  over  the  radio  carpal  joint. 

e.      Intercostal   Neuralgia 

This  localization  of  neuralgia  is  again  a  much  more  frequent  one.  Together 
with  the  causative  factors  for  neuralgias  in  general,  a  number  of  pathological 
conditions  of  the  thorax  and  its  contents  must  be  specially  mentioned;  such 
are  scoliosis,  kyphosis,  fractures  of  the  ribs,  pleurisies,  aortic  aneurisms. 
The  great  majority  of  cases,  however,  can  be  referred  to  general  deleterious 
influences  (infectious  diseases,  anemia,  cachexia,  etc.).  It  must  be  especially 
pointed  out  that  tumors  of  the  spinal  cord  for  a  long  time  may  have  their  true 
character  concealed  under  the  harmless  mask  of  an  intercostal  neuralgia.  As 
concerns  the  prognosis,  many  cases  of  intercostal  neuralgia  are  characterized 
by  extreme  persistence,  almost  all,  by  a  tendency  to  relapse.  The  pressure 
points  are  quite  characteristic:  the  "vertebral  point"  lies  just  along  the  spinal 
column  at  the  level  of  the  affected  nerve,  the  "posterior  perforating  point," 
upon  the  sternum,  or  the  rectus  abdominis,  close  to  the  middle  line. 

f.      Mastodynia 

Neuralgia  of  the  mammary  glands  or  mastodynia,  is  a  special  variety  of 
intercostal  neuralgia  which  is  limited  to  the  definite  branches  of  the  second 
to  sixth  intercostal  nerves  innervating  the  breast.  This  extremely  obstinate 
complaint,  which  is  identical  with  Astley  Cooper's  "irritable  breast,"  affects 
almost  solely  women,  often  appearing  for  the  first  time  during  pregnancy 
or  the  puerperium.  The  attacks  of  pain  sometimes  coincide  with  menstruation. 
The  skin  over  the  mamma,  especially  about  the  nipple,  is  extremely  hyperesthetic 
during  the  attack:  sometimes  also,  red  and  swollen.  Erb  has  described  the 
spontaneous  discharge  of  a  milky  fluid  at  the  height  of  the  attack  of  pain. 
Painful  points  are  the  vertebral  points  of  the  above  mentioned  five  intercostal 
nerves. 

g.     Lumbar  Neuralgia 

This  very  rare  form  of  neuralgia,  according  to  its  location,  is  divided  into 
lumbo-abdominal,  crural,  and  obturator  neuralgia.  One  should  be  on  his 
guard  in  these  cases  quite  as  much  as  in  brachial  neuralgias  against  overlook- 
ing local  causes  (lumbar  vertebra?,  tumors  in  the  pelvis). 


DISEASES    OF    THE    PERIPHERAL    NERVES  51 

h.     Sciatica 

Sciatica  neuralgia,  or  sciatica,  also  called  Cotugno's  disease,  after  the  classi- 
cal description  of  this  author  (1764)  is  the  most  frequent  of  all  the  forms  of 
neuralgia.  It  occurs  in  men  somewhat  oftener  than  in  women,  by  preference  in 
the  fourth  and  fifth  decades  of  life.  Its  causes  are  manifold,  particularly  often, 
exposure  to  cold,  as  by  sitting  on  cold  stones,  camping  on  wet  ground,  etc.,  are 
accused.  Also  trauma  plays  no  small  role.  This  sometimes  is  not  directly 
to  the  pelvis  but  may  affect  the  periphery  of  the  lower  extremity  (I  saw  a 
particularly  obstinate  case  arise  in  immediate  connection  with  stumbling,  the 
foot  being  caught,  through  which  the  limb  suffered  a  torsion).  On  account  of 
causing  compression  of  the  nerve  within  the  pelvis,  retrouterine  hematocele, 
chronic  constipation  with  fecal  impaction  in  the  rectum,  tumors  of  the  sacrum 
(carcinoma  metastases)  also  tuberculous  osteitis,  etc.,  act  as  causative  factors 
of  sciatica.  Quenu  has  also  called  attention  to  "varicose  sciatica"  in  conse- 
quence of  dilatation  of  the  veins  accompanying  the  nerves  to  the  hip.  All  these 
eventualities  must  be  considered  in  taking  the  history  as  well  as  in  estimating 
the  condition  (examination  by  rectum  or  vagina).  General  toxic  causes  which 
according  to  experience  often  form  a  substratum  for  sciatica,  are  particu- 
larly gout,  diabetes  (in  which  the  otherwise  very  rare — and  always  sugges- 
tive of  vertebral  disease — bilateral  sciatica  sometimes  occurs),  syphilis,  tuber- 
culosis, malaria,  typhoid  fever,  influenza,  gonorrhoea.  Finally  those  cases  which 
are  found  in  patients  with  flat  foot  or  with  diseases  of  the  generative  organs  or 
of  the  rectum  are  to  be  classed  as  reflex  neuralgias. 

Sciatica  can  begin  quite  suddenly  or  the  disease  picture  unfolds  itself  little 
by  little  to  its  full  height.  The  paroxysms  of  pain  are  either  separated  by  in- 
tervals of  entire  freedom,  or  (this  applies  to  the  majority  of  cases),  there 
persists  also  between  the  attacks  proper,  a  dull  feeling  of  pain  along  the  course 
of  the  affected  nerve  (in  the  region  of  the  loins  and  the  buttocks,  the  posterior 
surface  of  the  thigh,  in  the  popliteal  space,  on  the  outside  of  the  leg,  and  foot). 
The  pains  of  sciatica  are  increased  by  cold,  by  sitting  and  by  walking,  while 
lying  and  standing  are  in  general  most  tolerable.  However,  very  frequently  it 
night,  without  any  apparent  cause,  the  severest  paroxysms  occur. 

Vullcix's  points  are  sometimes  absent  in  otherwise  typical  eases  of  sciatica 
but  in  the  very  great  majority  of  cases  one  or  other  of  these  points  is  to  be 
recognized  as  sensitive:  1.  "Lumbar  point"  over  the  spinous  process  of  the  fifth 
lumbar  vertebra;  '2,  "Ueosacral  point"  near  the  posterior  superior  spine  of 
the  ilium;  .'5,  "Gluteal  points"  over  the  great  sciatic  foramen  and  in  the  told 
between  the  trochanter  and  the  tuber  ischii;  4,  "Popliteal  point"  in  the  middle 
of  the  popliteal  space;  •">.  "'Peroneal  point"  behind  the  head  of  the  fibula; 
<i.  ".Malleolar  point"  behind  the  external  malleolus  ;  7,  "Metatarsal  point"  in  the 
firsi  intermetatarsal  space  of  the  dorsum  of  the  foot.  The  gluteal  points  are 
most  frequently  recognizable. 

Of  great  diagnostic  importance  is  "Lasdgue's  sciatic  phenomenon."  While 
the  patient   rests  in  a  horizontal  position,  the  [eg  extended  at  the  knee,  is  -.lowly 

raised  by  the  examiner;  when  the  foot  becomes  separated  about  .'{•')  to  45  cm. 
above  the  hed,  there  is  sudden  and  severe  pain  on  the  posterior  surface  of  the 


52  LECTURE    III 

thigh  which,  however,  passes  away  as  soon  as  the  knee  is  bent  and  the  sciatic  is 
so  relaxed  again.  "Bonnet's  phenomenon"  depends  upon  the  same  principle; 
passive  simultaneous  flexion  of  the  hip  and  knee  joints  calls  forth  no  painful  re- 
action ;  this  occurs,  however,  when  the  leg  is  also  brought  into  the  position  of 
adduction. 

Finally,  in  only  part  of  the  severe  cases  of  sciatica  "Beehterew's  sciatic 
phenomenon"  is  found;  if  the  patient  extends  the  sound  leg  he  can  scarcely 
stretch  the  affected  one,  and  vice  versa. 

In  walking  a  patient  with  sciatica  instinctively  avoids  those  movements  which 
lead  to  stretching  the  affected  nerve,  and  on  this  account  generally  keeps 
the  hip  and  knee  of  the  side  involved  in  a  position  of  light  flexion.  Besides  this 
he  attempts  to  spare  the  affected  leg  as  much  as  possible  by  supporting  himself 
mainly  on  the  sound  one.  Also,  he  often  turns  the  foot  outward.  In  standing 
also  an  anomaly  of  position  is  often  observed;  in  order  to  relieve  the  affected 
leg,  by  letting  the  vertebral  column  fall  over  toward  the  healthy  side,  the  center 
of  gravity  is  shifted  toward  that  side.  There  is,  however,  also  a  form  of 
sciatica  (the  "spasmodic  sciatica"  of  Brissaud),  in  which,  by  a  contracture  of 
the  erector  spina;  on  the  affected  side,  occurring  reflexly,  a  homologous  scoliosis 
is  produced  instead  of  the  cross  scoliosis.  An  "alternating  sciatic  scoliosis,"  in 
which  the  crossed  and  the  homologous  scoliosis  alternate  with  one  another  has 
also  been  described.  With  cure  of  the  sciatica  the  anomalies  of  position  of  the 
vertebral  column,  as  a  rule,  entirely  disappear.  In  sitting,  the  sciatic  patient 
rests  his  weight  exclusively  on  the  sound  side,  he  "sits  on  the  edge  of  the  chair"; 
in  rising  he  avoids  bending  the  body  forward,  as  is  done  normally. 

The  objective  testing  of  sensibility  shows  either  no  anomalies,  or  hypesthe- 
sia  for  the  different  qualities  of  sensation  in  the  distribution  of  the  nerve  (most 
frequently  only  in  one  branch,  e.g.,  the  peroneal),  less  frequently  hyperesthe- 
sia. On  stroking  the  sole,  the  sensation  of  tickling  and  the  resulting  jerking 
away  of  the  foot  is  often  absent  on  the  affected  side,  while,  on  the  other  side 
both  are  present.  Motility  is  only  so  far  affected,  in  that  by  a  long  duration 
of  the  disease,  there  is  some  atrophy  of  the  affected  muscles,  and  that  during 
the  paroxysms  of  pain  sometimes  muscular  spasms  are  observed.  Herpes  is 
rare,  also  vasomotor  phenomena,  e.g.,  local  reduction  of  temperature.  The 
Achilles  tendon  reflex,  which  occurs  through  the  sciatic,  is  either  reduced  or 
absent,  or  (especially  in  Brissaud's  spasmodic  sciatica  already  mentioned),  on 
the  contrary  exaggerated.  The  same  remarks  apply,  as  a  rule,  to  the  patella 
reflexes  both  of  the  affected  and  of  the  sound  side.  Finally,  I  will  relate  two  more 
interesting  objective  symptoms  which  are  not  at  all  infrequent  in  severe  sciatic 
neuralgias ;  the  Achilles  tendon  on  the  affected  side  feels  more  relaxed  than 
upon  the  sound  side  (OppenJuim)  ;  the  gluteal  fold  hangs  lower  upon  the  af- 
fected side  and  shows  its  greater  flaccidity  sometimes  by  a  peculiar  folding 
{Bonnet). 

From  a  differential  diagnostic  point  of  view,  sciatic  neuritis  comes  specially 
into  consideration.  This  is,  as  compared  to  sciatic  neuralgia  characterized 
especially  by  continuity  of  the  pain,  greater  intensity  of  the  objective  disturb- 
ances of  sensibility  (sometimes  there  is  in  places  complete  anesthesia!),  more 
marked  muscular  atrophy,  finally,   reaction  of  degeneration,  more  prominent 


DISEASES    OF    THE    PERIPHERAL    NERVES  53 

vasomotor  and  trophic  anomalies.  In  addition  there  is  increase  in  the  size 
of  the  nerve  which  can  sometimes  be  felt  as  a  firm  cord  behind  the  trochanter. 
Very  frequently  chronic  sciatica  is  confused  with  malum  coxa?  senilis.  This 
confusion  can  be  avoided,  however,  if  it  is  remembered  that  Lasegue's  phe- 
nomenon is  absent  in  the  latter  disease,  while,  on  the  contrary,  movements  at 
the  hip  joint  are  more  difficult  and  painful,  that  pain  is  already  very  severe 
on  beginning  locomotion,  but  grows  less  in  the  course  of  walking;  that  further, 
in  contradistinction  to  sciatica,  it  is  dependent  upon  the  nature  of  the  road, 
is  increased  by  unevenness  of  the  ground,  and  that  finally,  not  the  nerve  trunk 
but  the  hip  joint  shows  itself  sensitive  to  pressure.  Not  adduction,  but  abduc- 
tion is  hindered,  thrusting  the-  head  of  the  femur  into  the  joint  cavity  (by  a  blow 
upon  the  trochanter)  calls  forth  a  sharp  pain  reaction. 

i.     Neuralgias   of  the  Feet 

The  foot  is  a  situation  of  predilection  for  certain  pains  limited  to  a  narrow 
region  and  of  plainly  neuralgic  character  whose  nature  is  often  obscure,  at 
whose  basis,  however,  there  is  usually  some  anomaly,  though  perhaps  a  trifling 
one,  of  the  neighboring  structures  (skeleton,  tendons,  etc.).  There  is  metatar- 
salgia  or  Morton  s  disease,  which  is  located  in  the  periarticular  nerve  branches 
of  the  fourth  metatarso-phalangeal  joint,  and  presumably  is  caused  by  an  ab- 
normally movable  fifth  metatarsal  bone  being  pushed  under  the  fourth  by  a 
too  narrow  shoe  and  there  exerting  pressure.  We  may  also  mention  talagia  or 
tarsalgia  (pain  in  the  heel),  for  which,  sometimes,  exostoses  on  the  calcaneum, 
or  diseases  of  the  serous  membranes  are  underlying  causes,  which,  however,  can 
be  observed  without  any  objective  findings;  the  same  is  true  of  Achillodynia, 
which  originates  from  the  point  of  insertion  of  the  Achilles  tendon. 

j.     Spermatic  Neuralgia 

This  form  of  neuralgia  is  characterized  by  exceedingly  severe  paroxysms 
of  pain  which  radiates  either  from  the  testicle  along  the  spermatic  cord  to  the 
inguinal  canal,  or  in  the  opposite  direction  and  is  almost  always  unilateral. 
At  the  same  time  there  is  great  hyperesthesia  of  the  scrotum  and  testicles,  so 
tli.it  even  a  slight  touch  is  very  painful  ("irritable  testis"  of  the  English). 
In  making  this  diagnosis  one  must  not  forget  to  think  of  local  diseases  of  the 
testicle  as  well  as  renal  colic,  in  which  the  pain  usually  radiates  into  the  testi- 
cles. We  will  learn  further,  on  considering  tabes  dorsalis,  that  in  this  disease 
of  the  spinal  cord  also,  pains  in  the  testicles  occurring  in  paroxysms  and  of 
very  severe  character,  may  be  observed. 

K.       COCCYGODYNIA 

In  COCCygodynia,  or  neuralgia  of  the  coccyx,  attacks  of  pain  either  occur 
spontaneously  or  are  set  up  on  sitting  down,  by  defecation,  or  by  walking. 
Pressure  points  are  found  over  the  coccyx.  As  a  chief  causal  factor  for  this 
disease,  which   attacks   females   by   preference,  difficult    labors   are   mentioned. 


54  LECTURE    III 

Traumatic  or  inflammatory  lesions  of  the  coccyx  or  of  the  muscles  inserted 
into  it,  usually  form  the  basis  of  the  disease,  however. 


D.     Treatment  of  Diseases  of  the  Peripheral  Nerves 

1.     THE    THERAPY    OF    PERIPHERAL    PARESES 
AND    PARALYSES 

We  will  refrain  from  considering  at  all,  at  length,  things  like  primary  nerve 
suture  in  the  severing  of  peripheral  nerves,  the  removal  of  tumors  which  com- 
press nerves,  the  separation  of  nerves  from  scars  or  cicatrices  and  masses  of 
callus,  all  of  which  you  will  find  described  in  surgical  works.  All  the  more 
important  will  it  be  for  us  to  undertake  a  careful  description  of  the  relations 
in  those  cases  of  interruption  of  conduction  which  present  no  indication  for 
the  procedures  indicated  (and  they  represent  the  impressive  majority). 

In  the  early  stage,  indeed,  too  active  therapy  should  be  avoided,  the  affected 
extremity  should  be  put  at  rest — in  a  middle  position  for  the  upper,  in  a  suit- 
able one  for  the  lower  extremities.  For  example,  if  in  a  peroneal  paralysis 
we  do  not  want  to  furnish  the  opportunity  for  development  of  a  pes  equinovarus, 
the  pressure  of  tiie  bedclothes  must  be  kept  off  the  feet ;  supporting  the  covering 
upon  a  frame,  however,  often  causes  in  patients  an  unpleasant  sensation  of  cold 
in  the  legs  unless  a  thin  layer  of  felt  is  spread  over  them.  In  order  that  the 
feet  be  kept  as  near  as  possible  in  a  position  at  right  angles  to  the  legs  we 
furnish  them  with  a  suitable  support  which  may  be  improvised  with  the  aid 
of  a  footstool,  a  cigar  box,  or  a  pasteboard  box.  Absolute  immobilization  of 
limbs  with  neuritis  (in  a  padded  wire  splint,  for  instance),  I  would,  in  general, 
advise  against,  and  would  prefer,  rather,  comparative  immobilization  by  placing 
them  between  sandbags ;  this  method  permits  greater  individualization  in  the 
matter  of  this  important  postulate,  to  relieve  tension  in  the  affected  nerve  as 
much  as  possible  and  to  protect  it  from  pressure.  In  musculo-spiral  paralysis, 
to  avoid  overextension  of  the  extensors  of  the  hand,  the  latter  must  not  be 
allowed  to  remain  permanently  in  a  position  of  "wrist-drop" ;  here  the  appli- 
cation of  a  small,  well-padded  dorsal  splint  will  make  possible  more  rapid  return 
of  contractility  in  the  muscles  supplied  by  this  nerve. 

In  the  second  place  it  is  important  to  keep  in  mind  the  general  condition 
of  nutrition  of  the  patient,  since  the  better  this  is,  the  more  reason  have  we 
to  hope  for  conditions  favoring  the  regeneration  of  the  nerve  and  the  return 
of  its  ability  to  conduct.  This  indication  is  frequently  a  "causal  indication," 
in  so  far  as  we  can  find  decided  anemia,  marantic  conditions,  glycosuria,  etc. 
In  alcoholic  neuritis  and  polyneuritis  absolute  prohibition  of  alcohol  is  in  place. 
An  exception  is  only  to  be  made  when  the  condition  of  the  heart  gives  reason  to 
fear  sudden  collapse  upon  complete  withdrawal,  or  if  the  psychical  condition  is 
such  as  to  arouse  suspicion  of  threatening  delirium.  In  such  cases  the  dose  of 
alcchol  is  to  be  reduced  as  far  as  is  considered  safe  and  complete  withdrawal  is 
to  be  gradually  accomplished. 

After  about  fourteen  days,  if  we  feel  sure  that  the  motor  symptoms  no  longer 


DISEASES    OF    THE    PERIPHERAL    NERVES  55 

show  a  progressive  tendency  (and  if  the  continuance  of  severe  pains  does  not 
suggest  further  abstention — see  below,  page  57  et  seq.),  the  aim  of  our  therapy 
should  be  energetic  support  of  the  process  of  repair. 

Electrotherapy  must  here  be  mentioned  as  our  most  valuable  agent;  when 
possible  applied  daily.  The  indifferent,  large  electrode  is  placed  upon  the 
chest  or  back  of  the  patient,  the  "different"  or  "stimulating"  electrode,  about 
10  cm  square,  successively  upon  each  of  the  paralyzed  or  paretic  muscles,  in 
which  at  first  5  to  10,  later  20  to  40  contractions  should  be  produced.  Rule: 
The  weakest  current  necessary  to  produce  a  decided  effect  should  be  used,  and 
as  different  electrode  that  pole  to  which  the  muscle  reacts  best  (also  usually, 
on  account  of  the  reaction  of  degeneration,  first  the  anode,  later,  as  there  is 
improvement,  the  cathode).  Faradization  of  the  muscles  is  usually  useless  in 
this  stage,  since  they  do  not  generally  react  to  the  induced  current;  in  later 
stages  of  convalescence  it  may  (in  the  form  of  faradic  massage  with  the  roller 
electrode)  be  in  place.  Used  too  early,  strong  faradization  in  neuritis,  and 
especially  in  polyneuritis,  may  be  directly  injurious  by  reawakening  pain  and 
by  unsuitable  application  (stimulating  the  antagonists)  may  favor  contrac- 
tures. That  in  spite  of  this,  unfortunately,  in  peripheral  paralysis  faradism 
is  far  too  much  used,  is  due  to  the  fact  that  many  physicians  are  obsessed  by 
the  opinion  that  electrization  consists  in  planlessly  running  over  the  parts  with 
the  faradic  electrode,  and  hence  neglect  to  provide  themselves  with  galvanic- 
apparatus  or  to  learn  its  use. 

Mild  massage  (with  avoidance  of  too  firm  petrissage)  will  also  be  of  ad- 
vantage in  the  stage  of  repair  of  peripheral  interruptions  of  conduction.  Par- 
ticularly important,  however,  is  the  intelligent  and  conscientious  application 
of  passive  movement  as  prophylactic  against  contractures  and  faulty  positions 
of  the  joints.  They  may  be  advantageously  applied  in  or  after  a  warm  bath, 
which  finally,  with  a  gradually  increasing  content  of  sea  salt,  brine,  pine  needle 
extract,  or  other  coniferous  preparations  is  usually  found  strengthening  and 
agreeable.  It  is  also  encouraging  for  the  patient  to  note  the  increased  ease 
with  which  he  can  move  the  paretic  limbs  in  the  bath  ("kinetotherapeutic 
baths");  in  this  factor,  which  depends  on  the  relative  reduction  in  weight 
through  the  buoyant  effect  of  the  water,  there  is  a  welcome  reeducatory  influ- 
ence. In  exercise  therapy,  which  is  best  undertaken  in  connection  with  electro- 
therapeutic  applications,  the  greatest  stress  should  be  laid  upon  movements 
against  graduated  resistance.  In  these,  the  patient  is  encouraged  to  exert  con- 
tractions of  the  muscles  against  moderate  resistance  made  by  the  physician, 
which,  separating  the  origin  and  the  insertion  of  the  muscle,  stretches  this,  and 
so  facilitates  its  voluntary  contraction.  It'  apparatus  for  half  active  (that 
is,  with  the  aid  of  machinery},  gymnastics  is  available,  it  can  be  used  with 
advantage. 

We  can,  and  should  also,  support  the  reparative  processes  with  drugs. 
Strychnin,  by  increasing  the  reflex  irritability  in  the  cells  of  the  anterior  horn 
of  the  spinal  cord,  favors  "path  formation"  by  the  motor  impulses.  Resides 
this,  it.  as  well  as  certain  phosphorus  derivatives  (glycero  phosphate  of  cal- 
cium, phvtin,  etc.),  Works  as  a   tonic,   increasing  the  appetite,  perhaps  Specially 

favors  the  nutrition  of  the  nervous  system.     It   can  he  {riven  as  tablets  con- 


56  LECTURE    III 

taming  grin.  0.001  (grain  1/60)  of  strychnin  nitrate,  one  or  two  twice  daily, 
or  as  tine.  nuc.  voni.,  10  drops  t.  i.  d..  or,  finally,  as  injections  of  strychnin 
nitrate  0.001 — 0.004  (gr.  1/60 — gr.  1/15)  once  a  day.  Finally,  arsenical 
medication  may  come  into  question  (Pil.  Asiatic,  Sol.  Fowler,  Sol.  Pearson, 
injections  of  Cacodylate  of  Sodium,  etc.). 

Finally,  if  after  peripheral  paralyses,  power  is  so  far  regained  that  the 
question  of  a  sojourn  at  a  health  resort  can  be  considered  (in  which  case 
besides  the  baths,  other  curative  factors  must  be  available  to  the  patient), 
we  have  the  choice  between  the  different  kinds  of  resorts,  among  which  the  selec- 
tion should  rest  more  upon  the  financial  resources  of  the  patient,  the  time  of 
year,  and  the  agreeability  of  the  resort,  than  upon  a  specific  difference  of  char- 
acter of  the  springs.  There  come  into  consideration:  1.  Saline  baths  (for 
example,  Rheinfelden,  Kreuznach,  and  in  the  United  States  Saratoga  and  Mt. 
Clemens,  Mich.).  2.  Carbonic  acid  salt  baths  (Nauheim,  Byron  Hot  Springs, 
California).  3.  Thermal  waters  of  indifferent  composition  (Teplitz,  Ragaz, 
the  Hot  Springs  of  Virginia  and  of  Arkansas).  4.  Thermal  waters  containing 
common  salt  (  Baden-Baden,  Wiesbaden).  5.  Hot  Sulphur  baths  (  Aix-les-Bains, 
and  in  the  United  States,  Clifton  or  Richfield  Springs,  New  York,  Glenwood 
Hot  Springs,  Colorado,  and  Gilroy  Hot  Springs,  California).  6.  Mud,  peat 
and  fango  baths  (Franzenbad,  Postyen,  Las  Vegas  Hot  Springs,  N.  M.,  Paso 
Robles,  California).* 

The  symptoms  which  remain  in  spite  of  the  different  therapeutic  measures 
which  we  have  described  are  to  be  treated  by  the  orthopedic  surgeon  with 
apparatus  for  extension,  or  for  support,  by  tendon  and  nerve  transplantation, 
etc. 

2.     THE    TREATMENT    OF    ANESTHESIA 

Where  there  is  decided  reduction  of  sensibility  there  is  always  the  danger 
that  the  patient,  on  account  of  his  insensibility,  will  not  protect  himself  from 
injuries  which  can  cause  deep  wounds  tending  to  heal  very  slowly,  hence  care 
must  be  taken  that  hot-water  bottles,  poultices,  the  thermaphore,  etc.,  are 
not  applied  too  hot.  The  so-called  "neuro-paralytic  keratitis"  which  is  some- 
times observed  in  anesthesia  of  the  upper  branch  of  the  trigeminus  nerve,  is 
only  in  part  to  be  considered  as  a  trophic  disturbance  and  the  fact  that  the 
eyeball,  on  account  of  its  loss  of  sensation  is  much  exposed  to  trauma,  erosions, 
etc.,  plays  in  this  case  the  chief  role.  If  the  action  of  foreign  bodies  from  the 
outside  is  prevented  by  suitable  protecting  glasses  (which  also  prevent  the 
drying  of  the  epithelium,  caused  by  interference  with  the  tear  secretion),  this 
serious  complication  can  usually  be  prevented.  For  anesthetic  skin  areas,  under 
some  circumstances  "dry  faradization"  with  the  wire  brush  electrode  is  to  be 
recommended. 

*  Names  of  American  resorts  added  by  the  Translator. 


DISEASES    OF    THE    PERIPHERAL    NERVES  57 

3.     THE    TREATMENT    OF    NEURITIC    AND    POLYNEURITIC 

PAIN 

In  patients  with  fresh  acute  neuritis  or  polyneuritis,  our  first  duty  is  to 
help  the  patient  over  the  days  of  the  worst  pain.  The  different  "antineuralgics" 
exercise,  it  is  true,  an  individually  variable,  but  in  general  a  quite  satisfactory 
effect.  As  a  rule,  pyramidon,  0.3  to  0.5  (grs.  4  to  7,  t.  i.  d.),  works  most 
efficaciously,  some  patients,  however,  respond  better  to  a  mixture  of  the  other 
analgesics.  Since  a  change  of  remedies  may  be  necessary  from  time  to  time, 
I  will  give  a  few  combinations  which  have  proved  useful  to  me: 

I£      Acctanilidi 0.2      (gr.  3) 

Phenacetini    0.1      (gr.  \l/i) 

Quinine  valer 0.05    (gr.    J4) 

Vy      Exalgini , 0.1  ( gr.  V/2) 

Caffeini  citric 0.05  (gr.  H) 

Phenacetini   0.2  (gr.  3) 

Antipyrini 0.4  (gr.  6) 

I£      Phenacetini    0.6      ( gr.   8) 

Acetanilidi 0.3      (gr.   5) 

Codeini 0.04    (gr.   J/2) 

3J      Aspirini, 

Lactophenini    aa   0.5      (gr.  7J/2) 

Caffeini  citrici 0.1       ( gr.    V/2  ) 

It  will  be  will  to  prescribe  at  night  an  analgesic  hypnotic  combination,  for 
example,  Pyramidon,  0.3  (grs.  41/j)  ;  Veronal,  0.5  (grs.  7%),  or  Lactophenin, 

Trional,  aa  0.5   (grs.  7V>).     With  ordinary  hypnotics,  if  the  pain  is  severe, 
almost   nothing  is  accomplished.     Tin-  following  mixture  will  give  good  service: 

\f.       Potass,  bromide 10.0  (3iiss.) 

Chlorali  hydrati    5.0  (gr.   Ixxv) 

Antipyrini    3.0  ( gr.   xlv) 

Codeini  phosph 0.4  (gr.  vi) 

Aq.  menth.  pip    ad  150.0  (,z,v) 

.M.S.     Tablespoonful  at  night. 

The  injection  of  morphine  "or  of  one  of  its  modern  succedanea  in  neuritis 
and  polyneuritis  should  never,  or  only  wry  exceptionally,  be  resorted  to. 

When  the  period  <>f  severe  spontaneous  pains  has  been  overcome  so  thai 
these,  in  spite  of  intense  sensitiveness  to  pressure  which  persists,  occur  with 
less  intensity,  thai  is,  after  about  four  or  five  days,  the  application  of  heat 
nniy  be  begun;  particularly  applicable  are  apparatus  for  hot  air,  for  example. 

liirr'.s  boxes. 

By  the  application  of  the  so-called  "Phoenix"  ("Phenix  a  I'air  chaud"), 


58  LECTURE    III 

which  permits  an  intensive  thermotherapy  to  be  carried  on  in  bed  without  dis- 
turbing the  patient,  we  may  obtain  at  the  same  time  a  conveniently  regulated 
diaphoresis.  Encouragement  of  the  sweat  secretion  is  often  considered  as  of 
itself  a  curative  agent  which  acts  upon  the  cause  in  toxic  polyneuritis,  and  in 
any  event  contributes  to  the  alleviation  of  polyneuritic  pains.  The  duration  of 
tins  sort  of  a  sweat  bath  (to  be  given  once  a  day)  should  be  from  10  to  15 
minutes ;  c;i  refill  control  of  the  pulse  is  absolutely  necessary  where  the  heart 
is  not  entirely  intact.  When  the  treatment  with  hot  air  is  inapplicable  on  ac- 
count of  weakness  of  the  heart,  or  for  some  other  reason,  the  application  of 
moist  heat  (two  to  three  times  a  day  from  one-half  hour  to  one  hour)  is  proper, 
in  spite  of  its  inconveniences;  nevertheless  the  application  of  a  compress  which 
is  best  soaked  with  warm  mildly  stimulating  decoctions  (camomile,  matricaria) 
as  well  as  the  envelopment  of  the  part  with  waterproof  tissue,  is  the  more 
disagreeable  for  the  patient  the  larger  the  skin  surface  which  must  be  treated. 
Linseed  meal  poultices  are  often  unpleasant  on  account  of  their  weight,  the 
same  is  true  of  moist  compresses  kept  warm  by  the  thermaphore;  the  dry 
thermaphore  is  often  found  disagreeable. 

During  this  time  we  should  endeavor  to  dispense  with  the  analgesics  pre- 
scribed at  the  start,  or  at  least,  only  to  order  them  at  night.  I  often  substi- 
tute for  these  drugs  aconitin  or  colchicin,  which  are  withdrawn  as  soon  as  the 
curative  effect  of  the  warm  applications  is  plainly  evident.  Aconitin  is  best 
given  once  or  twice  a  day  in  the  form  of  1/10  nig  (gr.  1/600),  taking  care  to 
use  an  active  preparation  (Merck's  or  dill's  preparation),  colchicin  in  the 
form  of  tinctura  colchici,  5  drops  t.  i.  d.  (eventually  tinct.  aconiti,  tinct. 
colchici,  aa  10  drops  t.  i.  d.). 

The  withdrawal  of  the  internal  analgesics  is  facilitated  by  some  external 
applications,  which  are  to  be  recommended  upon  psychological  grounds,  since 
they  occupy  and  divert  the  patient.  Massage  (also  the  rubbing  in  of  oint- 
ment) is  to  be  avoided  as  long  as  the  acute  painful  stage  is  not  certainly  over; 
also  any  stimulating  electric  application.  On  the  other  hand  the  stabile  treat- 
ment of  the  nerve  trunks  with  the  anode  (application  of  the  electrode  upon  the 
place  of  greatest  sensitiveness  to  pressure  for  from  3  to  5  minutes)  with  weak 
current  (3  to  5  milliamperes  an  electrode  of  the  size  of  a  dollar),  with  the 
cautious  turning  on  and  off  the  current  is  to  be  unqualifiedly  recommended. 
For  rubbing  on,  fluid  liniments  applicable  without  pressure  are  suitable.  Of 
these  the  following  are  examples: 

1.  01.  juniperi,  2.0;  ol.  terebinthini,  spts.  camphori,  spts.  saponis,  liq. 
amnion,  caust.,  aa.,  12.0.     (Shake  well.) 

2.  Camphor,  chloral  hydrat,  aa.,  20.0. 

3.  Chloroform,  10.0;  liq.  amnion.,  40.0. 

4.  Yeratrin.,  1.0;  chloroform,  alcohol,  aa.,  24.5  (cave  oculos). 

Upon  areas  which  are  the  seats  of  annoying  paresthesia,  pieces  of  flannel 
soaked  in  the  following  solution  can  be  laid  on:  Menthol,  guaiacol,  aa.,  1.0; 
alcohol  abs.,  20.0  (cave  oculos). 


DISEASES    OF    THE    PERIPHERAL    NERVES  59 

4.     THE    TREATMENT    OF    NEURALGIAS 

In  every  fresh  neuralgia  of  any  considerable  intensity,  bodily  rest  is  an 
urgent  requisite ;  in  trigeminal  or  intercostal  neuralgia,  as  a  rule,  keeping  to 
one's  room  ( in  which  case  visits,  etc.,  are  to  be  avoided)  is  sufficient.  In  sciatica, 
rest  in  bed  should  be  enforced.  In  this  the  painful  limb  should  be  carefully 
placed  in  such  a  position  as  relaxes  the  affected  nerve  as  much  as  possible. 
This  is  best  accomplished  by  placing  a  roll  of  felt  under  the  bend  of  the  knee  ami 
keeping  the  extremity  in  a  position  of  moderate  abduction  with  sand  bags. 
Sometimes  (in  varicose  sciatica,  for  example)  it  is  desirable  to  raise  the  foot 
of  the  bed.  A  patient  with  sciatica  should  not  leave  his  bed  until  the  neuralgic 
paroxysms  have  ceased,  or  at  least  have  plainly  abated.  He  should  be  told  in 
advance  that  it  will  be  necessary  to  remain  in  bed  two  or  three  weeks,  perhaps 
longer:  at  the  same  time  antineuralgic  remedies  should  be  given  and  in  suffi- 
cient doses — a  point  on  which  I  lay  considerable  stress,  since  many  failures  are 
due  to  too  timid  medication.  In  sciatica,  salicylate  of  sodium  should  always  be 
tried  first.  This  has  a  decidedly  more  energetic  effect  than  aspirin.  This 
last  is  to  be  preferred  only  in  people  with  sensitive  stomachs. 

I£  Sodii  salicylat,  20.0  (5v)  ;  syr.  aurantii  cort.,  -10.0  (3x)  ;  aq.  menth. 
pip.,  ad  300.0  (§x).  M.S.  A  tablespoonful  4  times  a  day  in  half  a  glass  of 
water  after  meals.  To  avoid  disturbance  of  the  stomach  a  solution  of  about  30 
grains  of  sodium  bicarbonate  can  be  taken  after  the  salicylate. 

The  daily  dose  can  soon  be  reduced  to  from  two  to  three  grammes  (30  to 
4o  grs.)  a  day.  As  to  the  other  antineuralgics,  I  would  refer  to  the  prescrip- 
tions recommended  for  neuritic  and  polyneuritic  pains.  Particularly  in  tri- 
geminal and  occipital  neuralgia  some  other  drugs  are  applicable,  sometimes 
with  quite  happy  effects:  Migranin  (citrate  of  antipyrin  and  caffein),  in  sin- 
gle doses  of  grin.  1.00  (15  grs.),  butylchloral  or  its  combination  with  pyra- 
midon.  trigemin  (which  must  only  be  used  when  it  is  of  a  clear,  white  color), 
in  single  doses  0.5  (71/2  grs.),  atropin  or  methylatropin,  the  first  in  doses  of 
0.0005  (gr.  1/120),  of  the  last  0.002  (gr.  1  30)  ;  finally  tinct.  gelsemii,  10  to 
15  drops.  Aconitin,  already  mentioned  in  the  treatment  of  neuritis,  is  also 
frequently  a  very  efficacious  remedy  in  trigeminal  neuralgia,  especially  when  it 
is  used  for  a  long  time  with  a  daily  dose  of  saline  laxative. 

The  thermotherapy  of  neuralgia  is  the  same  as  that  already  indicated  for 
neuritis  and  polyneuritis.  In  the  treatment  of  sciatica,  especially  in  subchronic 
and  chronic  cases,  besides  this,  the  following  procedures  are  suitable:  Electric 
lij;ht  hat  lis,  hot  air  douches,  sun  bat  lis,  strain  douches,  "FangO-packs,"  hot 
baths.  Cold  is  in  general  only  found  agreeable  in  fresh  cases  of  facial  neuralgia, 
and  in  these  may  even  work  curatively;  however,  on  this  point  there  are  so  great 
individual  differences  that  it  is  necessarj  to  try  it  out  from  case  to  case. 
Freezing  the  skin  over  the  Valleix  points  with  the  chloride  of  ethyl  spray  used 
for  local    anesthesia   deserves   special   mention.      It   can   he   tried   in   all    forms   of 

neuralgia  with  plainly  localized  sensitiveness  to  pressure  (in  the  neighborhood 
of  the  exes  great  caution  is  necessary;  the  eyes  must  be  carefully  covered  with 
cotton).      Naturally    the   freezing  of   the   skin    areas,   which    is   made   evident    hy 


60  LECTURE    III 

their  white  color,  can  only  last  a  few  seconds ;  otherwise  there  is  danger  of 
circumscribed  necrosis.  After  the  freezing  the  affected  part  must  be  carefully- 
rubbed  with  some  simple  ointment.  This  application  can  only  be  repeated  after 
the  reddening  of  the  affected  part  (which  sometimes  persists  long  after  the 
application  of  the  chloride  of  ethyl)  has  disappeared  and  the  parts  look  normal 
again.  Other  "revulsives,"  whose  application  is  naturally  restricted  for  the 
most  part  to  the  trunk  and  extremities,  are  blistering  with  cantharides,  paint- 
ing with  iodine,  dry  cups,  mustard  plasters,  capsicum  plasters,  faradization  with 
the  wire  brush,  ignipuncture,  etc.,  also  local  bleeding  (with  wet  cups  or  leeches) 
is  occasionally  applied.  In  all  these  applications  care  should  be  taken  that  the 
integument  should  not  be  injured  to  an  extent  which  would  prevent  the  later  ap- 
plication of  the  electric  current. 

In  galvano-therapy  the  stabile  application  of  the  anode  to  the  pressure 
points  is  to  be  preferred.  In  persistent  neuralgias  the  stabile  application  to 
the  diseased  nerve,  the  two  electrodes,  of  about  the  size  of  a  dollar,  being  placed, 
one  upon  the  most  proximal  and  the  other  upon  the  most  distal  point  in  the 
course  of  the  nerve,  which  is  accessible,  a  current  of  about  five  milliamperes 
gradually  introduced  and  allowed  to  pass  for  from  five  to  ten  minutes  (whether 
its  direction  is  ascending  or  descending  is  a  matter  of  indifference),  can  be 
used.  In  fresh  cases  of  neuralgia,  massage,  apart  from  light  vibration  of  the 
painful  points  is  to  be  avoided ;  in  old  cases,  on  the  other  hand,  particularly  in 
sciatica,  petrissage  of  the  affected  region  is  an  important  curative  measure, 
particularly  when  properly  carried  out  vibration  of  the  nerve  trunk  is  added  to 
it.  In  sciatica  also,  "bloodless  stretching"  of  the  nerve  is  a  mechanical  thera- 
peutic procedure  much  to  be  recommended;  it  is  especially  easy  to  carry  out. 
The  leg,  extended  at  the  knee,  is  raised  from  the  bed  as  in  Lasegiie's  test  (see 
above,  page  51),  but  only  so  far  as  it  will  go  without  provoking  any  consid- 
erable pain,  and  is  held  for  several  minutes  in  this  position;  at  the  end  of  this 
time  it  is  stretched  somewhat  more  strongly,  until  severe  but  still  tolerable  pain 
is  produced,  and  then  carefully  laid  down  again.  It  will  often  be  noticed  that 
from  one  treatment  to  another,  the  distance  through  which  the  leg  can  be  bent 
without  pain  increases.     "Bloody  nerve  stretching"  is  now  quite  abandoned. 

A  useful  procedure  is  the  injection  treatment  of  neuralgia.  We  distinguish: 
1.  Langc's  method  of  perineural  infiltration.  This  is  a  modification  of 
Schleich's  infiltration  anesthesia.  In  the  trigeminus  a  few  cubic  centimeters  of 
the  following  solution:  Beta-eucaine,  0.1;  normal  salt  solution,  100.0 — are  in- 
jected over  Valleix's  points  in  the  immediate  neighborhood  of  the  affected  nerve 
branch.  A  stovain-adrenalin  solution  according  to  the  following  formula  is 
also  used:  IJ  Sol.  adrenalin  (1%),  gtts.  v-x;  stovain,  0.1-0.2;  sol.  sodii 
chloridi  (0.8%),  ad  100.00.  In  the  sciatic  large  quantities  (70-100  cc.) 
are  injected.  A  Schleich  infiltration  is  made  over  the  point  of  exit  of  the 
nerve  (in  the  middle  of  a  line  joining  the  trochanter  and  tuber  ischii)  and  a- 
10  cm.  long  cannula  is  passed  carefully  through  this  down  into  the  nerve  (which 
is  here  1%  centimeters  in  diameter)  when  a  sensation  of  pain  (and  often  a  con- 
traction of  the  muscles  of  the  leg)  announces  that  the  cannula  has  entered  the 
nerve.  The  injection  is  then  made  by  means  of  a  syringe  or  an  irrigator. 
Whether  the  use  of  ice  cold  solutions  gives  better  results  is  questionable,  how- 


DISEASES    OF    THE    PERIPHERAL    NERVES  61 

ever,  the  action  of  simple  physiological  salt  solution  without  the  addition  of  an 
anesthetic  sometimes  is  very  satisfactory.  The  therapeutic  effect  of  this  pro- 
cedure is  perhaps  purely  mechanical;  a  swelling,  perhaps  a  stretching  of  the 
nerve  which  can  then  produce  a  cure  by  reactive  inflammation  is  its  result, 
accoi-ding  to  Lavge. 

2.  Neurolytic  injections.  In  these  methods  we  endeavor  to  injure  the 
nerve  by  the  injection  of  different  solutions,  also  in  a  way,  to  resect  it  chem- 
ically. We  reserve  them  on  this  account  for  severe  cases,  since  they  have  as  a 
result  anesthesia  of  the  skin,  and  avoid  them  in  mixed  nerves  (for  example,  the 
sciatic)  in  order  not  to  risk  a  motor  paralysis.  Further,  they  should  not  be 
made  into  the  supraorbital  canal,  since  this,  in  many  individuals,  communicates 
witli  the  orbit,  the  penetration  into  which  of  neurolytic  solutions  might  se- 
verely injure  the  optic  nerve.  We  will  not  discuss  all  the  substances  which  have 
been  applied  as  neurolytics  (ether,  carbolic  acid,  silver  nitrate,  chloroform, 
etc.),  but  will  only  indicate  two  solutions  from  which  we  have  sometimes  seen 
good  results:  (a)  1%  osmic  acid  solution;  (b)  80%  alcohol,  with  the  addition 
of  an  anesthetic  ("Schlosser's  injections").  Formulas  to  be  recommended  are: 
Alcohol  (80%),  20  cc;  menthol,  0.4 ;  novocain,  0.2;  or  alcohol  (80%),  20  cc. ; 
stovain,  0.2.  In  the  branches  of  the  trigeminus,  one  to  one  and  a  half  cc.  are 
injected  into  the  nerve,  if  possible,  or  at  least  into  its  immediate  neighborhood ; 
OstmiU,  indeed,  seeks  these  branches  directly  upon  the  base  of  the  brain  (in  the 
foramen  ovale  or  the  foramen  rotundum),  for  which  he  uses  a  specially  bent 
(bayonet  formed)  cannula,  passing  it  from  the  mouth;  Levy  and  Baudoin  pass 
the  needle  in  front  of  the  coronoid  process  of  the  lower  jaw,  or  between  this  and 
the  articular  process  through  the  cheek.  These  methods,  however,  have  not 
been  universally  accepted.  In  every  injection  of  such  solutions,  the  penetration 
of  a  vessel  must  be  carefully  avoided  by  first  inserting  the  empty  needle  and 
drawing  out  its  contents.  The  injection  of  osmic  acid,  a  drop  at  a  time,  into 
the  nerve  branch  laid  bare  by  operation,  and  indeed,  from  the  exposed  base 
of  the  skull  into  the  Gasserian  ganglion,  has  been  carried  out.  This  brings 
us  to  the  surgical  therapy  proper  of  prosopalgias.  The  extra  cranial  methods 
are  easiest;  simple  section  (neurotomy)  has  been  given  up,  since  rapid  reunion 
incurs,  and  for  it  has  been  substituted  nerve  evulsion  after  Thiersch-Witzel 
(neurexaresis)  which  permits  a  tearing  out  of  the  affected  nerve  from  the  base 
of  the  skull  to  its  terminal  ramifications.  In  spite  of  this,  many  prosopalgias 
which  have  been  subjected  to  neurexaresis  recur,  as  do  many  cases  treated 
with  neurolytic  injections.  While,  however,  the  last  can  lie  repeated  a  number 
of  times,  after  evulsion  of  all  three  branches  of  the  trigeminus,  the  trouble 
iniist  1m  attacked  at  its  root,  in  the  fullest  sense  of  the  word  and  extirpation  of 
I  lie  Gasserian  ganglion  must  he  performed.  Fedor  Krause,  the  originator  of 
this  operation,  states  that  in  sixteen  years  he  has  performed  it  sixty-four  limes 
and  has  never  observed  a  recurrence.  In  two  cases,  which  I  know  from  my 
own  observation,  recovery  also  occurred.  However,  this  severe  operation 
temains  a  last  resource,  in  the  fortunately  rare,  specially  obstinate  cases  of 
prosopalgia. 

In   severe   intercostal   neuralgias    the   corresponding   posterior   roofs    have 
been  divided  with  good  effect.     Neurectomy  has  been  done  not  so  infrequently  in 


62  LECTURE    III 

occipital  and  spermatic  neuralgias.      In  the  X-ray  treatment  of  neuralgias  I 
have  had  no  personal  experience. 

Finally,  it  must  be  remarked  that  a  change  of  diet  to  a  purely  egg,  milk 
and  vegetable  regime,  can  be  of  the  greatest  use  in  neuralgia,  and  that  of  course 
the  treatment  of  any  recognizable  underlying  disease  must  never  be  neglected 
(iron,  quinine,  mercury,  iodide  of  potassium,  etc.),  that  in  all  senile  forms  of 
neuralgia  an  arsenic  cure  should  not  be  left  untried,  and  finally,  that  the  bal- 
neotherapy of  neuralgias  is  entirely  similar  to  that  recommended  for  neuritis. 


LECTURE    IV 
The  Dyskinesias 

Gentlemen  :  In  neurology,  abnormal  motor  processes  which  occur  in  part 
as  symptoms  accompanying  different  diseases  of  the  nervous  system,  in  part 
are  to  be  considered  as  autonomous  pictures  "sui  generis,"  play  an  important 
and  interesting  role.  In  this  lecture  and  in  the  next  one  we  will  occupy  our- 
selves with  these  "Dyskinesias." 

A.     Tremor 

As  tremor  we  designate  involuntary  rhythmic  oscillatory  movements,  which 
affect  now  all  the  muscles,  again  only  single  groups.  According  to  the  rapidity 
of  the  oscillations,  we  speak  of  rapid  or  slow  tremor  (the  extremes  are  about 
between  4  and  10  oscillations  per  second),  according  to  the  amplitude  of  the 
excursions,  of  coarse,  medium  and  fine  tremor.  The  coarsest  form  is  denom- 
inated tottering,  the  finest  as  vibrating  tremor  or  thrill.  If  the  tremor  is 
absent  during  rest,  appearing  only  upon  carrying  out  movements,  it  is  called 
intention  tremor.  A  particular  kind  of  tremor  of  the  fingers  is  the  so-called 
Quinquaud's  phenomenon,  which  was  formerly  considered  a  pathognomonic 
symptom  of  chronic  alcoholism,  but  can  occur  in  all  disease  conditions  of 
which  tremor  is  a  symptom.  If  the  examiner  presses  his  hands  against  the 
tips  of  the  spread  fingers  of  the  patient,  he  will  perceive  in  these  a  peculiar 
unrest  in  the  articulations,  in  which,  presumably  on  account  of  a  tremor  of 
the  interossei,  the  joint  ends  of  the  phalanges  are  pulled  from  side  to 
side.  Within  normal  limits,  tremor,  as  is  well  known,  occurs  on  shivering,  in 
great  fatigue,  and  in  the  emotion  of  apprehension;  also  the  tremor  of  old 
age,  as  long  as  it  remains  within  moderate  bounds,  may  be  considered  as  a 
physiological  phenomenon;  with  pathological  tremor  of  the  most  varied  origin, 

on    the   other   hand,   we   will    meet    ill    the    further   COUrse   of   these    lectures,   quite 

frequently  as  a  symptom  of  many  functional  and  organic  nervous  diseases. 
A!  present,  however,  we  will  only  consider  that  form  of  tremor  which  in  itself 
constitutes  a  disease. 

ESSENTIAL    TREMOR 

In  this  disease,  which  occurs  mainly  as  a  hereditary  family  affection,  the 
single  symptom  is  a  rhythmical  tremor  of  small  amplitude,  but  without  char- 
acteristic tempo.   Nevertheless,  it   is  in  general  more  rapid  in  young  person-,. 

slower    in    old    ones.       It    ceases    during    sleep,    sometimes    also    while    the    patient 

is  awake,  hut  in  absolute  physical  ami  mental  rest;  in  any  event,  it  is  much 
less  marked  undi  r  these  la^l  conditions.     Many  patients  can  control  the  tremor 

63 


64  LECTURE    IV 

by  an  effort  of  the  will,  while  in  others  such  an  attempt,  on  the  contrary, 
increases  the  tremor.  The  carrying  out  of  voluntary  movements  can  arrest 
the  tremor ;  one  of  my  patients,  for  instance,  was  able,  by  strongly  clenching 
the  right  fist,  to  arrest  the  tremor  in  both  hands ;  on  the  other  hand,  how- 
ever, an  intentional  character  of  this  essential  tremor  is  occasionally  observed. 
All  the  voluntary  muscles  can  be  affected.  The  symptom  is  often  exclusively 
observed  in  the  hands.  Tremor  of  the  legs  can  sometimes  interfere  with  walk- 
ing, that  of  the  tongue  with  speech.  Frequent  complications  are  malforma- 
tions, epilepsy,  psychoses,  on  which  account  the  French  speak  of  "tremor  of 
the  degenerate."  Here  and  there  alcoholism  of  the  parents  has  been  accused 
of  being  an  etiological  factor;  nevertheless,  there  are  families  with  tremor  in 
which  the  ascendants  have  been  characterized  by  great  abstemiousness.  As 
exciting  causes,  overexertion,  strong  emotion,  infectious  diseases,  have  been 
mentioned.  The  disease  is  now  congenita],  again  it  begins  during  childhood  or 
puberty,  or  even  later.    The  fortieth  year  of  life  is  about  the  farther  boundary. 


ZM 


"P*^'   ^Z^<^^^X^^M4.1 


Fig.  27. 

Hereditary   Family   Essential  Tremor.      Handwriting. 

A.  Before  treatment.     B.  During  treatment. 

The  tremor  may  be  stationary,  may  progress,  or,  rarely,  may  grow  less,  but 
never  ceases  entirely.  Therapy  can  accomplish  but  little.  Regnault  claims 
to  have  obtained  improvement  by  limiting  the  amount  of  alcohol  used;  on  the 
other  hand,  in  a  family  with  tremor,  described  by  Ndgy,  the  persons  who 
drank  most  had  the  least  tremor.  In  the  particularly  severe  case  under  my 
own  observation,  whose  handwriting  is  shown  in  Fig.  27,  after  the  taking  of 
wine  the  tremor  much  decreased,  while  after  coffee  it  decidedly  increased. 
Bromides,  veronal,  adalin,  scopolamin,  were  without  influence,  while,  on  the 
other  hand,  the  patient  reacted  to  the  "pilula?  hyoscyami  composite"  (extr. 
hyosc.  zinc,  oxid.,  aa  5.0  (gr.  Ixxv)  ;  extract,  valer.  10.0  (oiiss).  M.  Fiat 
pil.  No.  100.  S.  3-5  per  day)  with  decided  improvement  of  the  tremor,  so 
that  the  formerly  indecipherable  handwriting  became  legible  again  (see  Fig. 
27). 

B.     Fibrillary  Contractions 

We  give  this  name  to  a  symptom  of  motor  irritation  which  manifests 
itself  in  isolated  and  successive  contractions  of  the  single  fiber-bundles  of  a 
muscle.  To  motor  manifestations  proper,  these  contractions,  which  are  usually 
of  lightning-like  rapidity  and  plainly  perceptible  both  by  inspection  and  pal- 


THE    DYSKINESIAS  65 

pation,  do  not,  as  a  rule,  lead,  though  occasionally  when  the  phenomenon  is 
very  marked  in  the  thenar  muscles,  a  slight  twitching  of  the  thumb  may 
result.  Fibrillary  contractions  occur  in  functional  neuroses  and  can,  indeed, 
be  provoked  in  normal  people  (for  example,  by  refrigeration)  ;  but  they  are 
of  most  importance  clinically  when  they  inform  us  of  a  pathological  process 
in  the  anterior  horns  of  the  spinal  cord  (see,  farther,  Lectures  VI  and  VII). 
A  variety  of  fibrillary  contractions  is  myokymia. 

C.     Muscular  Spasms 

In  the  large  group  of  symptoms  of  motor  irritation  (hyperkinesias)  which 
we  denominate  "cramps"  or  "spasms,"  two  categories  are  to  be  separated 
from  each  other:  tonic  and  clonic  spasms. 

Contractions  of  single  muscles,  or  of  muscle  groups,  of  short  duration 
repeating  themselves  in  fits  and  starts  are  characteristic  of  clonic,  pro- 
tracted contractions  of  tonic  spasm.  By  a  succession  of  clonic  and  tonic 
spasms  the  complicated  irritative  phenomena  denominated  as  convulsions 
arise.  These  we  will  have  to  study  more  closely  under  epilepsy.  They  con- 
stitute the  most  important  symptom  of  motor  irritation  of  cerebral  origin. 
Also  by  the  action  of  pathological  irritants  upon  the  spinal  motor  tracts  in 
the  lateral  columns,  there  occur  sometimes  spasmodic  phenomena ;  for  example, 
the  so-called  Brown-Sequard's  spinal  epilepsy,  a  spontaneous  clonic  twitching 
occurring  in  attacks,  a  more  or  less  severe  shaking  of  the  affected  extremities 
on  account  of  alternating  contractions  of  their  extensors  and  flexors.  The 
spasm-producing  properties  of  many  animal  and  vegetable  poisons  are  known 
to  you ;  for  instance,  those  of  the  toxine  of  tetanus  and  of  strychnine.  We 
will  not,  however,  enter  into  these  things,  but  will  turn  our  attention  to  the 
"idiopathic"  forms  of  spasm. 

1.     LOCAL    SPASMS 

As  to  the  etiology  of  these  conditions  we  are  very  poorly  informed ;  we 
can  only  say  that  neuropathic  individuals  and  those  afflicted  with  neuroses 
and  psychoses  are  predisposed  to  localized  muscle  spasms,  and  that  in  a  good 
part  of  the  cases  irritants,  working  reflexly,  appear  to  be  the  starting-point 
for  the  affection.  So,  for  example,  carious  teeth,  arthritis  of  the  jaw-joint, 
suppuration  of  the  jaw  cavities,  eye  diseases,  have  been  held  responsible 
for  spasms  of  the  .jaw  and  facial  muscles.  Only  seldom  can  a  disease  process, 
(Forking  as  a  direct  irritant  upon  the  nerves  of  the  muscles  affected  with 
spasm,  lie  discovered:  for  example,  in  facial  spasm,  aneurism  of  the  vertebral 
artery,  gumma  at  the  base  of  the  brain,  or  caries  of  the  petrous  bone,  etc. 
The  most  important  types  of  localized  muscle  spasm  are: 
1.  Masticator//  Spasm. — While  tonic  spasms  of  the  masticatory  muscles 
(the  so-called  lock-jaw  or  trismus)  play  a  notable  clinical  role  as  a  symptom 
of  tetanus,  of  meningitis,  and  of  other  general  diseases,  as  a  purely  local 
disease  manifestation  they  are  rare,  occurring  most  frequently  in  connection 
with  inflammatory  lesions  of  the   jaws.     Clonic  spasm  is  somewhat   more  fre- 


6() 


LECTURE    IV 


quent,  a  gnashing  of  the  teeth  occurring  in  the  form  of  attacks  at  more  or 
less  short  intervals.  The  prognosis  is  in  general  favorable.  Oppenheim  recom- 
mends in  the  treatment  of  fresh  cases  in  which  a  rheumatic  influence  may  be 
in  action,  diaphoresis;  further,  some  derivative  procedure  (cantharidal  plaster 
over  the  temples  or  upon  the  mastoid  process,  eventually  the  actual  cautery 
to  the  back  of  the  neck),  sedative  drugs,  and  the  galvanic  current. 

2.  Mimic  cramp,  or  facial  spasm,  one  of  the  most  frequent  locations  of 
peripheral  spasm,  is  usually  clonic ;  a  tonic  facial  spasm  is  the  cause  of  the 
peculiar  facial  expression  in  tetanus,  which  has  been  called  "liisus  sardonicus" 
(see  Fig.  28).  It  seldom  affects  all  the  facial  muscles,  but  is  usually  restricted, 
either  to  the  muscles  of  the  mouth  and  chin  (Orbicularis  oris,  Levator  anguli 


Fig.  sjb 
"Risus  Sardonicus"  in  Traumatic  Tetanus. 


oris,  Mentalis,  Zygomaticus),  or  to  the  Orbicularis  palpebrarum,  in  which 
latter  case,  when  the  spasm  is  tonic,  we  speak  of  blepharospasm.  The  expres- 
sions nictitation,  spasmus  nictitans,  and  blepharoclonus  denote  specially  the 
clonic  forms  of  lid  spasm.  With  the  exception  of  spasms  of  the  lids,  which 
in  many  cases  are  relatively  easy  to  treat  and  to  cure  permanently,  the 
prognosis  of  facial  spasm  is  in  general  not  good,  since  it  is  an  obstinate 
complaint  and  even  after  it  is  relieved  recurrence  is  to  be  feared.  Further, 
there  are  unfortunately  many  cases  which  cannot  be  cured  and  the  patient  must 
then  choose  between  the  facial  spasm  and  an  incurable  facia]  paralysis;  that 
is,  he  must  permit  a  neurectomy  or  neurexaresis.  In  these  cases,  also,  in 
patients  hereditarily  neuropathic,  one  is  never  sure  that  the  spasm  may  not 
later  attack  some  other  nerve.  As  regards  the  therapy  of  facial  spasms, 
apart   from   the  last   resort   above   mentioned,    pathological    conditions    which 


THE    DYSKINESIAS  67 

might  set  up  the  trouble  by  reflex  influences  should  be  removed  (diseases  of 
the  eye,  nose,  accessory  sinuses,  and  ears,  caries  of  the  teeth,  retained  wisdom 
teeth,  etc.).  In  the  way  of  medication,  a  trial  of  bromide  of  potassium  in 
daily  doses  of  at  least  -i  grms  (oj),  or  of  scopolamin,  twice  daily,  0.0005  (gr. 
1/120)  is  justified.  Unfortunately,  these  medicines  only  exceptionally  help. 
Edingcr  recommends  antipyrin  (in  combination  with  bromide  of  potassium). 
I  have  never  seen  any  result  from  this  in  facial  spasm.  From  electrical  treat- 
ment stabile  applications  the  anode  over  the  trunk  of  the  facial  nerve,  or  the 
whole  pes  anserinus  (under  the  general  rules  mentioned  for  the  treatment 
of  neuritis,  on  page  55),  I  have  seen — with  sufficient  patience  and  persistence 
on  the  part  of  patient  and  doctor — occasionally  the  best  results  in  spasm 
of  the  orbicularis  oris,  only  exceptionally,  however,  any  favorable  influence 
worth  mentioning  in  the  other  localizations  of  facial  spasm.  Still  another 
electro-therapeutic  procedure  comes  into  consideration  in  blepharospasm,  the 
stabile  application  of  the  anode  to  a  recognizable  "pressure  point"  (that  is, 
in  some  place  from  which  an  attack  of  spasm  can  be  cut  short  by  firm  com- 
pression, acting  reflexly)  ;  this  is  usually  one  of  the  already  mentioned  Valleix 
points  in  the  region  of  the  ophthalmic  division.  When  the  above-mentioned 
methods  fail  one  must  turn  to  the  neurolytic  injections  (after  Schlosser), 
which  have  been  described  at  length  under  the  treatment  of  neuralgia.  Eighty 
per  cent,  alcohol  with  a  local  anesthetic  (see  formulae  on  page  61)  is  injected 
through  a  cannula  inserted  between  the  external  auditory  canal  and  the  mas- 
toid process  in  the  neighborhood  of  the  stylomastoid  foramen  indicated  by  the 
styloid  process;  that  is,  into  the  facial  nerve  at  its  point  of  exit.  If  the 
injection  is  successful  a  facial  paralysis  which  lasts  for  several  months  is  pro- 
duced; if  this  is  not  to  be  obtained,  this  result  is  reached  by  exposing  the 
nerve  and  stretching  it  vigorously  (pulling  it  up  with  a  "squint  hook").  In 
favorable  cases  after  the  facial  paralysis  has  recovered,  spasm  does  not  return; 
unfortunately,  there  are  cases  enough  which  have  recurred  both  after  alcohol 
injections  and  after  nerve  stretching. 

To  be  distinguished  from  facial  spasm  is  the  psychogenic,  isolated  "tic 
facial"  of  the  French  writers  (Brissaud,  Meige,  Feimlcl),  which  is  a  twitching 
or  grimacing  which  has  become  a  habit,  which  has  the  character  of  voluntary 
movement,  and  is  to  be  treated  and  cured  by  exercises.  The  object  of  these 
exercises  is  gradually  to  carry  out  definite  movements  witli  the  facial  muscles 
affected  willi  tie  upon  command,  later  to  the  heat  of  a  metronome;  between 
these,  however,  to  keep  the  face  still.  I. .iter  the  patient  is  allowed  to  practice 
complicated  and  more  continued  series  of  movements  of  head,  neck,  and  face 
niiisrlrs  until  these  follow  quietly  without  being  interrupted  by  spasms.  All 
these  exercises  are  to  be  earned  out  before  a  mirror,  When  there  is  an 
emotional  basis  for  facial  tic,  Dubois  has  accomplished  cures  by  pure  psycho- 
therapy. 

.'5.  Spasm  of  tin-  Tongue  (Glossospasm). —  Isolated  spasms  of  the  tongue 
are  excessively  infrequent.  They  can  he  tonic  and  clonic.  As  reflex  causes 
setting  them  oil',  inflammatory  affect  ions  of  the  buccal  cavity  have  been  espe- 
cially mentioned.  The  prognosis  is  usually  not  had.  Therapeutically  stabile 
galvanization,    the    anode    on    the    hvpoglossus,    is    particularly    recommended; 


68  LECTURE    IV 

in  a  case  of  Lange's  this  nerve  also  was  stretched  and  later  resected,  but  only 
division  of  the  genioglossal  muscles  effected  a  definite  cure. 

4.  Spasm  of  the  Pharyngeal  Muscles  (Pharyngism). — When  this  form  of 
spasm  docs  not  arise  upon  the  basis  of  a  general  disease  of  the  nervous  system, 
for  example,  hydrophobia  or  tetanus,  or  is  not  an  expression  of  a  local  organic 
disease  (retro-pharyngeal  abscess,  carcinoma  of  the  oesophagus,  a  foreign 
body,  e.g.,  a  fish-bone  in  the  mucous  membrane),  it  is  usually  a  symptom 
of  hysteria. 

5.  Spasm  of  the  (Esophagus  (CEsophagism) . — To  this  phenomenon  the 
remarks  made  regarding  pharyngism  equally  apply.  After  tonic  spasms  of 
the  lower  parts  of  the  oesophagus  or  of  the  cardiac  orifice  of  the  stomach, 
dilatations  of  the  upper  part  of  the  oesophagus  have  been  observed. 

(3.  Spasm  of  the  Larynx  (Laryngismi). — This  form  of  spasm  is  not  at  all 
infrequent  in  children,  particularly  in  rachitic  infants.  We  will  later  have 
the  opportunity  of  referring  to  its  relation  to  the  tetany  of  infants.  It 
occurs  in  the  form  of  tonic  contraction  of  the  muscles  closing  the  glottis 
(thyroarytenoids  and  interarytenoids)  coming  on  in  attacks  which  may  lead 
to  severe  dyspnoea  and  cyanosis,  indeed,  to  asphyxia.  Well-known  popular 
remedies  like  douching  the  child  with  cold  water,  the  production  of  vomiting 
by  tickling  the  back  of  the  throat,  etc.,  sometimes  suffice  to  cut  short  the 
attack;  on  the  other  hand,  tracheotomy  may  be  necessary.  Along  with  the 
treatment  of  rickets,  which  is  often  present,  the  effect  of  the  different  internal 
sedatives  is  usually  very  satisfactory,  so  that  the  prognosis  in  general  may 
be  said  to  be  favorable.  To  be  recommended  is  a  prescription  of  Babinski: 
K<  Potassii  bromid,  1.5-3.0  (gr.  20-4.5);  tinct.  moschi,  1.0-2.0  (gr.  15-30); 
syr.  simp.,  15.0  (Yi  oz.)  ;  aq.  dest.,  q.s.,  ad  100.0  (3  ozs.).  M.S.,  a  dessert- 
spoonful every  two  hours.  Reflex  spasm  of  the  larynx  as  a  result  of  laryn- 
gitis occurs  both  in  children  and  adults. 

7.  Spasms  of  the  muscles  of  the  throat,  neck  and  shoulders  are  not  very 
frequent,  but  are  usually  characterized  by  great  persistence.  They  occur 
nearly  always  in  neuropathic  individuals  ;  exciting  causes  are,  in  the  psychical 
sphere,  fright;  depressive  emotions,  etc.;  in  the  somatic,  local  trauma,  dis- 
eases of  the  vertebral  column  or  of  its  ligaments,  etc.  Not  infrequently  its 
etiology  is  obscure.  We  distinguish  different  forms  of  spasm  which  are  quite 
characteristic  in  their  manner  of  occurrence. 

Tonic  unilateral  spasm  of  the  sternocleidomastoid  is  called  spasmodic  tor- 
ticolis  (Caput  obstipum  spasticum)  ;  in  this  the  patient  turns  the  head  and 
raises  the  chin  toward  the  sound  side,  while,  on  the  affected  side,  ear  and 
shoulder  are  approached  to  one  another.  The  sternocleidomastoid,  on  the 
side  on  which  the  head  is  drawn  down,  stands  up  under  the  skin  like  a  firm 
cord  (see  Fig.  29).  This  affection  is  not  to  be  confused  with  the  harmless, 
temporary  stiff  neck  due  to  a  rheumatic  affection  of  the  sternocleidomastoid; 
characteristic  of  this  latter  is  the  decided  painfulness  of  the  muscle  upon 
pressure  and  in  passive  movement.*      Clonic  unilateral  spasm  of  the  sterno- 

*  I  once  saw,  however,  in  a  patient  with  hysterical  predisposition,  a  spasm  of  the  sterno- 
cleidomastoid develop  as  a  sequel  to  a  rheumatic  stiff  neck. 


THE    DYSKINESIAS 


69 


cleidomastoid  proceeds  in  the  form  of  attacks  of  twitching  through  which  the 
head  is  pulled  by  fits  and  starts  into  the  position  described  above.  Spasm  of 
the  sternocleidomastoid  is  usually  combined  with  one  of  the  trapezius,  which 
is  also  supplied  by  the  spinal  accessory  nerve ;  the  head  then  is  either  clonically 
thrown  backward  or  there  is  a  tonic  "retrocollis."  Rotary  spasm  of  the 
head  depends  upon  clonic  contractions  in  the  obliquus  capitis  inferior;  nodding 


Fin.  29. 
Spasmodic  Torticollis. 


spasm  (spasmus  nutans)  (called  also  "salaam  spasm"  after  the  method  of 
salutation  of  the  Orientals)  upon  those  of  the  deep  muscles  of  the  neck  (rectus 
capitis,  longus  colli,  etc.),  usually  with  involvement  also  of  the  sternocleido- 
mastoids. This  last-mentioned  form  of  spasm  is  observed  especially  in  children, 
and,  according  to  Kassowitz,  is  always  due  to  rickets,  which,  however,  other 
pediatrists  deny.  The  following  mainly  tonic  spasms  should  be  mentioned: 
spasm  of  the  splenius,  in  which  the  head  is  drawn  backward  toward  the 
affected  side  and  at  the  same  time  is  somewhat  rotated,  while  laterally  from 
the  cervical  portion  of  the  trapezius  the  contracted  muscle  stands  plainly 
out;  spasm  of  the  rhomboid,  which  draws  the  scapula  into  an  oblique  position 
so  that   its   median   border    runs   oblique    from    below    and   within    to    above   and 


70  LECTURE    IV 

without ;  finally,  the  rare  spasms  of  the  Levator  anguli  scapulae,  Platysma 
myoides,  and  Omohyoid. 

In  connection  with  treatment  I  would  recommend,  after  correction  of  what- 
ever causal  factor  can  he  found,  next  to  take  care  that  the  patient  is  isolated 
for  several  weeks  in  order  to  reduce  external  irritants,  particularly  psychical 
ones,  to  a  minimum.  He  should  be  allowed  to  see  no  one  except  those  with  whom 
he  is  entirely  familiar,  and  about  whom  he  does  not  have  to  trouble  himself, 
besides  the  doctor.  Psychotherapeutically,  one  should  untiringly  endeavor  to 
keep  the  patient  placid  and  confident.  In  addition,  bromide  of  potassium 
should  be  given  in  daily  doses  of  3  or  4  grammes  (grs.  45-5 j )  (best  in  one 
dose  in  the  morning),  eventually  combined  with  grm.  1.0  (gi's.  15)  of  anti- 
pyrin.  Also  scopolamin  hydrobrom.,  0.0005  (gr.  1/120)  twice  a  day  is  worth 
trying.  At  the  same  time  the  stabile  application  of  galvanism  with  the  anode 
and  a  current  gradually  raised  to  3-5  milleamperes  should  be  used.  Points 
of  application:  1.  Pressure  points.  2.  The  motor  points  of  the  affected 
muscles  (see  Fig.  3,  page  25).  3.  Those  of  the  nerve  trunk  in  question. 
In  connection  with  this,  I  am  accustomed  to  faradize  the  antagonists.  Spring- 
supporting  apparatus,  with  a  head  holder  which  opposes  the  pathological 
anomaly  of  position  in  tonic  spasm  of  the  neck  muscles,  sometimes  aids  our 
therapy  decidedly ;  one  of  my  patients,  a  machinist,  very  intelligently  con- 
structed such  a  thing  for  himself.  I  would  decidedly  warn  you  against  rigid 
apparatus  (plaster,  etc.),  in  spite  of  its  recommendation  by  StriimpeU.  Op- 
penhcim  recommends,  further,  the  application  of  derivatives  at  the  back  of 
the  neck  (blistering  plaster,  a  hair  seton,  and  particularly  the  actual  cautery)  ; 
I  am,  however,  no  great  friend  of  these  procedures.  If  isolation,  psycho- 
therapy, drugs,  galvanization,  etc.,  supported  by  careful  stretching  massage 
and  prolonged  tepid  baths,  are  without  effect,  neurolytic  injections  are  to  be 
considered,  which,  however,  in  order  not  to  injure  the  important  neighboring 
structures  should  only  be  made  into  the  exposed  nerves  of  the  muscles  affected 
with  spasm;  however,  it  is  more  rational  to  undertake  the  mechanical  rather 
than  the  chemical  injury  of  the  nerve;  that  is,  stretching  it  until  paresis  be- 
gins ;  neurectomy  is  the  next  most  radical  procedure.  It,  like  myotomy  of 
the  muscles  affected  with  spasm,  has  found  more  partisans  among  the  surgeons 
than  among  the  neurologists,  who  have  often  after  the  operation  seen  the 
spasm  pass  over  to  neighboring,  up  to  this  time  sound,  muscles. 

Even  the  Kocher-de  Quervain  operation,  in  which  successively  the  follow- 
ing muscles  are  cut :  Trapezius,  Splenius,  Sternocleidomastoid,  Complexus  and 
Obliquus  colli — with  subsequent  curative  gymnastics,  does  not  produce  a  cure 
in  every  case;  in  the  case  shown  in  Fig.  29,  which  did  not  react  to  other  methods 
and  in  spite  of  being  easily  hypnotizable,  not  even  to  hypnosis,  myotomy  in- 
deed, effected  a  cure. 

8.  Localized  Muscular  Spasms  in  the  Extremities. — These  are,  on  the 
whole,  quite  rare.  Tonic  forms  of  spasm,  sometimes  unilateral,  sometimes 
bilateral,  have  been  observed  among  others  in  the  following  muscles:  Pectoralis 
major,  Latissimus  dorsi,  Deltoid,  Biceps,  Supinator  Longus,  the  flexors  of  the 
fingers,  the  adductors,  Gastrocnemius,  and  Tibialis  anticus,  clonic  forms  in 
the  Ileopsoas   and   the  Peronei. 


THE    DYSKINESIAS  71 

Not  to  be  confused  with  these  true,  local  spasmodic  conditions  of  the  mus- 
cles of  the  extremities,  are  the  painful  cramps  which,  as  is  well  known,  occur 
after  fatiguing  marches,  long  swimming,  mountain  climbing,  etc. ;  also  in 
healthy  people,  in  the  calf  muscles  (less  frequently  in  the  Abductor  hallucis, 
Quadriceps,  Tibialis  anticus)  and  for  which  certain  individuals  have  an  ex- 
aggerated predisposition  so  that  relatively  slight  exertion  suffices  to  set  up 
cramp.  In  contradistinction  to  true  clonic  spasms,  cramp  is  relieved  by  mas- 
sage and  passive  stretching. 

9.  Spasm  of  the  diaphragm  in  tonic  form  is  very  rare  and  is  almost  never 
a  disease  in  itself;  it  is  most  frequently  observed  in  tetanus.  All  the  more 
familiar  is  clonic  spasm  (singultus)  which  occurs  in  many  healthy  people,  now 
reflexly  (as  from  drinking  strong  spirits),  again  without  apparent  ground 
as  an  occasional  temporary  and  entirely  harmless  phenomenon.  Obstinate 
pathological  forms  of  singultus  occur  in  diseases  of  the  cervical  region  of  the 
cord  (in  the  neighborhood  of  the  diaphragm  center)  further  in  peripheral 
irritation  of  the  phrenic  nerve  (aortic  aneurism,  mediastinitis,  pericarditis, 
pleurisy,  etc.),  occasionally,  also,  as  an  apparently  idiopathic  and  localized 
spasm.  Familiar  popular  means  of  cutting  short  singultus  (holding  the 
breath,  sipping  cold  water,  etc.)  fail  in  severe  forms.  Energetic  faradization 
of  the  region  of  the  stomach  with  the  wire  brush  often  succeeds  here.  Other 
derivatives  to  be  applied  in  this  last  location  are  sinapisms,  blisters,  igni- 
puncture,  chloride  of  ethyl  spray.  Of  internal  sedatives,  besides  bromide  of 
potassium,  opium  is  recommended.  Lubordc  has  introduced  rhythmical  trac- 
tions of  the  tongue,  which  sometimes  succeed.  Finally,  the  galvanization 
(anodal)  of  the  phrenic  nerve  comes  into  consideration. 

Complicated  respiratory  spasms  which,  indeed,  only  occur  in  the  hysteri- 
cal, are  yawning  spasm,  sneezing  spasm,  screaming  spasm,  snoring  spasm. 
These  are  of  psychic  origin,  '■tics.''  like  voluntary  movements,  not  true  spasms, 
and  their  treatment,  mutatis  mutandis,  has  to  he  carried  out  from  the  points 
of  view  considered  under  facial  tic.  We  must  still  mention  that  there  is  a 
diseased  condition  characterized  by  the  most  manifold  motor  automatisms 
which  has  been  described  as  "Myospasia  convulsiva,"  "Maladie  des  tics,"  or 
''general  tic."  This  occurs  chiefly  in  individuals  of  neuropathic  constitution 
beginning  in  childhood,  and  is  often  accompanied  with  psychic  disturbances; 
for  example,  the  compulsory  ejaculation  of  certain,  sometimes  senseless,  often 
obscene  or  blasphemous,  words  (Koprolalia).  Often  there  is  also  the  com- 
pulsion to  repeat  words  heard  or  gestures  seen  (Echolalia.  Echopraxia). 
There  are  very  severe  incurable  cases  of  this  disease.  In  one  mild  case  which 
I  saw,  cure  resulted  from  a  "mast  cure,*'  combined  with  the  most  rigid  isolation 
in  a  dark  room,  to  which,  later,  a  course  of  re-education  of  movements  was 
added;  in  other  cases  considerable  improvement;  these  were  in  children.  The 
older  the  individual  the  worse,  in  general,  the  prognosis.  Drugs  and  physical 
therapeutics  are  useless,  so  is  hypnosis.  Similar  to  myospasia  convulsiva, 
but  of  favorable  prognosis,  usually  recovering  spontaneously  after  weeks  or 
months,  is  the  "saltatory  reflex  spasm"  described  by  Bamberger,  in  which  the 
patients  on  standing  break  into  jumping  movements,  while  on  sitting  .and 
lying  they  hold  themselves  quite  normally. 


72  LECTURE    IV 


2.     "OCCUPATION    SPASMS" 


From  the  disease  pictures  summed  up  under  the  name  of  "Local  Muscular 
Spasms"  we  separate  a  pathogenetically  unique  nosological  group  which  is 
distinguished  by  its  great  practical  importance.  Its  members  have  been  called 
"occupational  spasms,"  also  (after  Benedikt)  "co-ordinatory  occupation  neu- 
roses," and  had  already  been  recognized  and  their  clinical  peculiarities  noted 
by  Bell  and  Duchcnne,  namely,  as  spasms  which  appear  only  in  connection 
with  a  definite  activity  acquired  by  practice.  They  usually  appear  not  only 
as  spasms  (which  practically  always  are  of  tonic  character),  but  not  at  all 
infrequently  as  tremor,  and  occasionally  as  a  refusal  to  act  on  the  part  of 
the  affected  muscles,  which  last  it  has  been  attempted  to  separate  off  as  the 
"paralytic"  variety  of  the  occupation  neuroses.  As  a  diagnostic  criterion 
the  limitation  of  these  different  dyskinetic  disturbances,  not  only  to  definite 
motor  acts,  but  also  to  the  muscle  groups  acting  together  in  this  function, 
is  of  decisive  importance.  For  the  occurrence  of  this  affection  two  factors  are 
necessary:  1.  A  neuropathic  disposition  (usually  hereditary).  2.  The  fre- 
quent repetition  of  the  act  in  question  (usually  through  the  carrying  out  of 
one's  calling).  That  along  with  these,  alcoholism,  nephritis,  chronic  lead 
poisoning,  and  a  number  of  other  general  injurious  influences  contribute  to 
the  production  of  occupation  spasms,  is  made  probable  by  a  number  of  striking 
observations.  Of  local  predisposing  factors,  diseases  of  the  tendon  sheaths 
(as  ganglion),  farther,  exostoses  on  the  bones,  muscular  and  articular  rheu- 
matism, have  been  mentioned.  As  exciting  causes,  somatic  and  psychic  trau- 
mata have  been  especially  frequently  determined. 

The  best-known  example  of  occupation  spasm  is  writer's  cramp  ("crampe 
des  ecrivains,"  graphospasm).  It  occurs  usually  either  on  beginning  writing, 
or  only  after  some  lines  or  pages  have  been  written,  as  a  tonic  contraction 
of  the  flexors  of  the  fingers ;  much  less  frequently  there  is  spasm  of  the 
extensors  or  abductors ;  occasionally  the  hand  begins  to  tremble  or  the  fingers 
lose  power  and  let  fall  the  pen ;  cramplike  pains  can  occur  also.*  Other 
occupation  spasms  of  the  upper  limbs  are:  Milker's  cramp,  described  in  1851 
by  BasedoTc;  farther,  piano  player's,  telegrapher's,  shoemaker's,  drummer's, 
tailor's,  cigarmaker's,  violinist's,  'cellist's,  flutist's,  seamstresses',  sawyer's, 
newspaper  folder's,  smith's,  zither  player's,  and  watchmaker's  cramps.  In  the 
legs  there  occur  ballet-dancer's  cramp ;  in  the  muscles  of  the  mouth  and  tongue, 
trumpeter's  and  clarinetist's  cramps.  Oppcnhclm  has  described  a  "shaving 
cramp"  ("keirospasm"  or  "xyrospasm").  The  most  "modern"  spasms  are 
"daktylographer's"  and  "automobilist's"  cramps. 

These  occupation  neuroses  are  not  to  be  confused  with  the  professional 
pareses — which  are  mainly  of  neuritic  nature — already  mentioned.  I  have, 
however,  seen  develop  in  a  cigarmaker,  a  year  after  recovery  from  a  typical 
cigar-roller's  cramp,  a  not  less  typical  cigar-roller's  paresis,  with  atrophy  of 
the  small  muscles  of  the  hand,  which  then,  after  rest  and  later  change  of 
occupation,  entirely  disappeared.     This  last,  on  account  of  the  great  obstinacy 

*  For  the  vasomotor  form  of  "writer's  cramp"  see  Lecture  XXV. 


THE    DYSKINESIAS  73 

of  many  occupation  spasms,  is  not  very  infrequently  the  end  result  of  vain 
therapeutic  endeavors.  A  considerable  number  of  the  cases,  however,  are 
fortunately  not  refractory  to  the  means  of  treatment  at  our  disposal. 

First,  the  specific  harmful  occupation  must  be  entirely  given  up  for  a  period 
of  weeks,  during  which  time  complete  therapeutic  repose  is  to  be  sought  for 
in  the  first  place,  since  anxiety  about  the  spasm  and  fear  as  to  the  eventuality 
of  having  to  give  up  one's  calling,  usually  greatly  depress  such  patients  and 
serve  to  keep  uj)  the  neurasthenia  which  furnishes  the  basis  of  the  occupa- 
tional neuroses.  Prolonged  tepid  baths,  Swedish  gymnastics,  stabile  anodal 
galvanization  of  the  nerves  and  muscles  in  question  (with  avoidance  of  all 
sudden  variations  of  current  strength),  a  sojourn  in  the  mountains  or  at  the 
seaside,  a  course  of  arsenic  or  iron,  are  to  be  prescribed,  according  to  cir- 
cumstances. Later,  the  patient  is  allowed  to  carefully  take  up  again  the  "crit- 
ical" occupation,  in  which  case  this  is  to  be  undertaken  in  the  most  rational 
and  easy  manner  possible;  writing,  for  instance,  with  a  soft,  not  too  sharp, 
steel  pen  in  a  thick  cork  penholder,  or,  even  better,  special  "writers'  cramp" 
penholders,  as,  for  example,  that  of  Zabludowski,  or  "Nussbaum's  bracelet," 
which  is  held  by  the  spread  fingers;  also  by  finding  out  the  easiest  position 
for  the  hand.  I  let  the  patient  at  first  go  over  for  a  few  minutes  at  a  time 
a  very  coarse  copy  placed  on  tracing  paper,  later  writing  it  smaller.  Little 
by  little  he  is  allowed  to  practice  longer,  and  finally  to  write  without  a  copy. 

For  patients  with  pianist's  cramp,  Zabludowski  recommends  practice  on 
a  juvenile  piano,  which  has  a  considerably  smaller  scale  than  the  regular 
instrument.  Telegraphists  should,  when  possible,  exchange  the  Morse  appa- 
ratus for  that  of  Hughes. 

3.     THE    MYOKLONIAS 

Under  this  name  we  include  some  rare,  pathogenetically  still  obscure,  dis- 
ease conditions  whose  common  chief  symptom  consists  in  clonic  contractions 
of  the  individual  muscles  which  usually  produce  but  little  movement,  or  none 
at  all.     Three  chief  forms  may  be  distinguished. 

1.  Paramyoklonus  multiplex  (Friedreich')  occurs  usually  about  the  fiftieth 
year  of  life,  sometimes  set  up  by  a  psychic  or  somatic  trauma,  infectious 
diseases,  excesses,  and  sometimes  without  any  recognizable  cause.  It  shows 
itself  in  attacks  lasting  a  minute  or  so  (of  irregular  rhythm  and  a  frequency 
of  10  to  15  per  minute)  of  lightning-like  contractions  of  different,  usually 
symmetrical  muscles  of  the  trunk  and  extremities;  for  example,  the  latissimus 
dorsi,  gastrocnemius,  quadriceps,  pectoralis,  rectus  abdominis,  biceps  brachii, 
triceps,  ete.  The  contractions  are  most  marked  during  rest  (in  sleep,  indeed, 
they  usually  stop,  or  at  least  grow  ]eSs)  ;  on  movement  they  are  less.  In  one 
case  I  described  a  coincidence  with  congenital  defect  of  the  pectoralis,  which 
is,  of  course,  to  be  considered  as  a  stigma  of  congenital  deficiency  of  the 
muscles. 

2.  Myoclonus  Epilepsy  (Unverricht) . — Symptomatologically  particularly 
Characterized  by  the  myoklonic  contractions  affecting  the  tongue,  the  phar- 
yngeal, and  diaphragmatic  muscles,  this  affection  appears  almost  always  in 


74  LECTURE    IV 

several  children  of  a  family,  not  rarely  in  several  successive  generations.  Note- 
worthy also  is  its  association  with  occasional  epileptiform  attacks  occurring 
particularly  at  night,  which  sometimes  show  themselves  as  precursors  of  the 
myoklonic  manifestations  (the  last  usually  begin  about  the  tenth  year  of 
life).  The  myoklonic  phenomena  increase  on  movement  and  in  psychical  ex- 
citement. The  tendon  reflexes,  as  well  as  the  mechanical  irritability  of  the 
muscles  and  nerves,  are  increased.  Life  is  seldom  directly  threatened  (for 
example,  by  aspiration  pneumonia  as  a  sequel  to  myoklonic  spasms  of  the 
pharynx).  The  patients  can  live  to  be  as  old  as  seventy  years,  in  which  case 
there  is  usually  gradual  mental  failure  and  termination  in  "Dementia  myo- 
clonica"  and  marasmus. 

3.  Nystagmus-myoclonia  (Lcnoble-Aubineau). — A  rare  affection,  almost 
exclusively  observed  in  the  Celtic  families  of  Brittany  and  Great  Britain,  in 
whom  as  a  congenital  and  stationary  condition  contractions  of  the  external 
eye  muscles,  as  well  as  of  the  extremities,  which  are  increased  by  cold  and  by 
tapping  the  muscles,  but  can  to  a  certain  extent  be  voluntarily  controlled,  have 
been  observed.  The  reflexes  are  usually  much  exaggerated,  different  trophic 
and  vasomotor  disturbances  (for  example,  deformities  of  the  teeth,  asymmetry 
of  the  face  and  the  body,  local  sweating,  circumscribed  oedema,  lividity  of  the 
skin)  are  also  found. 

Therapeutically,  stabile  galvanization  of  the  muscles  with  the  anode,  warm 
baths,  bromide  of  potassium,  chloral  hydrate,  can  be  used  in  the  myoclonias 
as  fulfilling  to  some  extent  the  symptomatic  indications. 

4.     TETANY 

As  characteristic  of  this  disease,  given  its  name  by  Corvisart,  we  can  con- 
sider tonic  spasms,  which  specially  affect  the  peripheral  portions  of  the  limbs, 
occur  in  attacks,  and  are  usually  combined  with  more  or  less  decided  pains. 

It  is  usual  to  separate  a  "primary"  tetany  from  the  different  "secondary" 
or  symptomatic  tetanies.  The  first,  whose  etiology  is  still  obscure,  attacks, 
as  a  rule,  previously  healthy  and  strong  males  between  fifteen  and  twenty-five 
years  old,  and  by  preference  those  carrying  on  some  definite  calling,  particu- 
larly shoemakers  and  tailors,  more  rarely  joiners  and  locksmiths.  Cold  and 
damp  weather  favors  the  onset  of  the  disease;  the  majority  of  cases  occur  in 
the  months  from  January  to  April.  The  geographical  distribution  of  the 
disease  is  striking;  it  is  most  frequent  in  the  neighborhood  of  Vienna  and 
Budapest,  while  it  is  quite  infrequent  in  France,  and  has  decreased  in  fre- 
quency from  decade  to  decade ;  in  Basle  and  its  neighborhood  tetany  of  workers 
is  practically  unknown.  I  sought  in  vain  for  years  for  a  case  and  have  had  no 
word  of  any  definite  observation  by  any  one  else,  a  fact  all  the  more  remark- 
able since,  in  the  not  very  distant  Heidelberg,  primary  tetany  is  compara- 
tively frequent.  The  "secondary"  tetanies  occur  in  the  course  of  different 
affections,  and  hence  are  grouped  as  follows:  1.  Gastrointestinal  tetany,  which 
is  occasionally  observed  in  profuse  diarrhea  or,  on  the  other  hand,  in  obsti- 
nate constipation ;  also  in  dilatation  of  the  stomach  and  stenosis  of  the  pylorus, 
in  carcinoma  of  the  stomach,  appendicitis,  intestinal  helminthiasis  and  chole- 


THE    DYSKINESIAS  75 

cystitis.  2.  Intoxication  tetany,  which  can  occur,  for  example,  in  ergotine, 
lead,  opium,  atropin,  and  alcohol  poisoning.  3.  Tetany  in  acute  infectious 
diseases;  this  is  occasionally  observed;  sometimes  in  the  beginning,  sometimes 
in  the  course  of  typhoid  fever,  dysentery,  measles,  cholera,  scarlatina,  influenza, 
diphtheria,  malaria,  acute  rheumatism,  etc.  -i.  Maternal  tetany,  which  devel- 
ops in  pregnant,  puerperal,  and  nursing  women,  and  was  first  described  by 
Trousseau  under  the  name  of  "contracture  rhumatismale  des  nourrices."  5. 
Parathyroid  tetany,  the  result  of  destruction  of  the  parathyroid  glands. 
It  is  not  long  since  tetany  occurring  after  extensive  or  total  thyreoidectomy 
was  regarded  as  due  to  the  loss  of  this  gland.  To-day,  however,  clinical  and 
experimental  proofs  have  been  furnished  by  Vassale,  Generali,  Moussu,  Pineles, 
Erdhcim,  de  Quervam,  Hagenbach,  Isdln,  and  others,  that  usually  the  loss 
of  the  accessory  glands  to  the  thyroid  provokes  the  onset  of  tetanic  phe- 
nomena. 6.  The  tetany  of  children,  which,  as  a  rule,  occurs  in  rachitic  children 
and  in  those  suffering  from  gastrointestinal  disturbances.  The  theory  that  all 
varieties  of  tetany  depend  upon  an  insufficiency  of  the  parathyroid  glands  is 
not  proved,  but  has  much  to  recommend  it ;  their  function  is  plainly  an  anti- 
toxic one;  it  can  be  conceived  that  tetany  occurs  not  only  when  the  para- 
thyroids are  destroyed,  but  also  if  their  ability  to  neutralize  exogenic  or 
endogenic  poisons  is  not  sufficient.  In  the  tetany  of  workers  and  maternal 
tetany,  hypothetical  toxines  entering  the  organism  or  produced  in  it  have  long 
been  spoken  of.  According  to  Ycmase,  however,  a  number  of  cases  of  the 
tetany  of  children  depend  upon  hemorrhages  into  the  parathyroids  (probably 
at  birth). 

The  symptomatology  of  tetany  is  characterized  by  a  number  of  exceed- 
ingly typical  phenomena.  Sometimes  (namely  in  primary  tetany)  there  is 
complaint  of  certain  prodromal  symptoms,  namely,  paresthesias  in  the  hands 
and  feet,  general  malaise,  muscular  unrest;  from  these  prodromes  patients 
who  have  already  had  tetany  are  able  to  recognize  the  oncoming  new  attack. 
Tin  se  last  are  entirely  dominated  in  their  clinical  aspect  by  the  attacks  of 
spasm  which  arc  usually  preceded  by  a  feeling  of  tingling  in  the  affected 
limbs,  tin-  so-called  "sensory  aura."  The  upper  extremities  are  most  fre- 
quently and  severely  affected,  particularly  the  hands,  whose  characteristic 
attitude  Trousseau  lias  described  ill  a  classical  manner.  The  thumb  is  brought 
into  a  forced  position  of  adduction,  the  closely  approximated  remaining  fingers 
are  bent  against  it  predominantly  at  the  metacarpophalangeal  joints;  also 
the  palm  of  the  hand  forms  a  hollow,  on  account  of  the  approximation  of  its 
radial  and  ulnar  borders,  and  the  whole  hand  takes  the  wedge  shape  assumed 
by  the  obstetrician  lor  vagina]  examination  ("accoucheur's  hand").  The 
wrist  is  tonically  flexed,  and  in  extensive  spasms,  the  elbow  also,  while  by  firm 
contraction  of  I  hi'  pectoralis,  the  upper  arm  is  pressed  against  the  thorax; 
only  exceptionally  does  the  arm  take  a  position  of  extension.  Extension  at 
the   hip   and    knee    joints,   on    the   contrary,    is    typical    in    tetanic    spasm    of   the 

legs;  the  feet,  however,  imitate  the  "obstetric  hand"  by  approximation  of  the 
maximally  flexed  toes,  under  which  the  overadducted  big  toe  is  forced,  hollow- 
ing the  sole.  In  severe  tetany,  tonic  spasms  may  affect  the  face  muscles; 
Escherich  has  called  at  lent  ion  to  a  trunklike  protrusion  of  the  lips  occurring 


76  LECTURE    IV 

in  this  case.  In  involvement  of  the  masticatory  muscles  trismus  occurs ;  in 
that  of  the  external  eye  muscles,  squinting ;  sometimes  the  long  muscles  of  the 
back  and  the  sphincters  are  also  affected.  Particularly  important  are  spasms 
of  the  glottis  which  for  a  long  time  were  regarded  as  the  privilege  of  infantile 
tetany,  which,  however,  Pineles  has  shown  are  not  so  infrequent  in  that  of 
adults.      The  spasms  of  tetany  are  usually  symmetrical,  but  one  side  can  be 


Fig.  30. 
Parathyroid  Tetany. — Position  of  the  hands  in  the  attack  (instantaneous  photograph). 

affected  before  the  other,  or  to  a  greater  extent.  As  to  the  length  of  the 
attacks,  they  vary  from  a  few  minutes  to  several  days ;  in  cases  of  the  last 
sort,  ulcers  have  been  known  to  be  produced  by  the  unintermitting  pressing 
together  of  the  fingers.  As  already  said,  more  or  less  manifest  painful  sensa- 
tions usually  accompany  the  tetanic  spasms. 

Besides  the  tonic  spasms  (which  when  they  are  set  up  by  voluntary  move- 
ment are  called  "intention  spasms"),  tetany  presents  a  number  of  exceedingly 
interesting  motor  phenomena,  which  occasionally,  in  rudimentary  or  "latent" 
tetany,  can  occur  without  an  attack  of  spasm;  on  the  other  hand,  they  need 
not  all  be  present  in  typical  tetany.  These  are  Trousseau's,  Clivostek's,  Erb's, 
and  the  Pool-Schlesingcr  phenomena. 


THE    DYSKINESIAS  77 

1.  Trousseau's  sign:  If  with  the  fingers  energetic  pressure  is  exerted  upon 
the  nerves  of  an  extremity  (especially  upon  the  nerves  of  the  upper  arm  in 
the  internal  bicipital  sulcus),  or  if  a'limb  is  firmly  bound  with  a  rubber  tube, 
after  a  few  seconds  or  minutes  a  typical  attack  of  spasm  occurs  in  it. 

2.  Chi'ostek's  sign:  Tapping  the  trunk  of  the  facial  nerve  in  front  of  the 
ear,  or  even  stroking  this  region,  sets  up  contractions  in  the  muscles  of  the  face. 

3.  Erb's  sign :  There  is  galvanic  ovcrexcitability  of  the  muscles  and  nerves. 
The  cathodal  closing  contraction  appears  with  a  very  weak  current  (in  the 
case  of  the  girl  shown  in  Fig.  30  with  one-eighth  milleampcre  on  the  ulnar). 
It  is  further  characteristic  that  the  anodal  contractions  (AnOC  and  AnCIC) 
can  be  obtained  with  a  comparatively  weak  current.  Finally,  according  to 
Mann  and  Thicmich  in  infantile  tetany,  and  according  to  Pineles,  sometimes 
in  that  of  adults  also,  it  is  typical  that  contraction  on  cathodal  opening 
occurs  below  milleampercs  and  that  AnOC  is  greater  than  AnCIC.  Faradic 
overirritability  is  rarer. 

4.  The  Pool-Schlesinger  sign  ("leg  phenomenon"):  If  in  the  time  between 
attacks  the  leg  extended  at  the  knee  is  flexed  at  the  hip  (or  if  the  patient 
lying  on  his  back  is  ordered  to  sit  up,  his  knees  being  kept  pressed  together), 
after  a  few  minutes,  or  often  after  a  few  seconds,  there  occurs  a  spasm  of 
extension  at  the  knee  and  a  tonic  spasm  at  the  ankle,  with  severe  pain.  The 
spasm,  as  a  rule,  is  limited  to  the  extremity  under  examination,  and  usually 
ceases  when  flexion  at  the  hip  is  relaxed. 

Galvanic  hyperexcitability  of  the  sensory  nerves,  occasionally  observed  in 
tetany,  is  known  as  Hoffman's  phenomenon;  the  supraorbital  nerve,  for  in- 
stance, reacts  to  0.2  instead  of  to  1.0  milleampcre.  According  to  Chvostek, 
Jr.,  and  v.  Franhl-Hoclncart,  galvanic  overexcitability  of  the  nerves  of  hear- 
ing and  taste  can  also  occur. 

It  remains  for  us  to  enumerate  a  number  of  rare  and  inconstant  symptoms 
of  tetany.  First,  along  with  the  tonic  spasms  there  occur  more  or  less  severe 
attacks  of  clonic  contractions  which  sometimes  are  accompanied  by  alteration 
of  consciousness,  are  "epileptiform";  this  is  most  frequent  in  the  tetany  of 
infants,  since  in  general  infancy  is  predisposed  to  eclampsia. 

Moderate  rise  of  temperature,  or,  on  the  contrary,  hypothermia  is  some- 
time observed.  Other  trophic  and  vasomotor  accompanying  phenomena  are 
a  certain  puffiness  of  the  face  which  in  primary  tetany  has  even  been  described 
as  the  ".tetany  countenance";  further,  there  is  sometimes  a  redness  or  cyanosis 
of  the  face  or  of  the  extremities,  oedema  of  the  hands  and  feet,  also  of  the 
joints,  falling  out  of  the  nails  and  hair,  and  finally  cataract.  Trophic  symp- 
toms are  in  general  characteristic  of  severe  chronic  relapsing  tetany.  Of 
psychic  phenomena,  hallucinatory  conditions  in  sequence  to  the  attacks  of 
Spasm   are   to   he   mentioned.      Tin    tendon   reflexes  are,   as   a    rule,  exaggerated* 

The  course  and  prognosis  of  tetany  vary  within  comparatively  wide  limits. 
Maternal  tetany  always  runs  an  acute  and  favorable  course;  also  cases  occur- 
ring in  infectious  diseases  and  intoxications  always  recover,  the  cause  ceasing 
to   act. 

In  parathyroid  tetany,  all  depends  upon  the  amount  of  uninjured  para- 
thyroid   substance    left;    total    ablation    of   these   bodies    causes    chronic    tetany. 


78  LECTURE    IV 

Gastrointestinal  tetany  is  a  severe  disease,  with  a  tendency  to  relapse  and 
to  pass  over  into  chronicity.  The  prognosis  depends  above  everything  else 
upon  whether  the  stomach  or  intestinal  affection  can  be  removed.  In  infantile 
tetany,  attacks  of  eclampsia,  or  spasm  of  the  glottis,  may  cause  death.  As 
to  primary  tetany,  Pineles  writes  that  the  prognosis  in  a  certain  portion  of 
the  cases  is  quite  favorable,  since  the  disease  is  recovered  from  in  a  longer  or 
shorter  time,  that  however,  in  a  not  inconsiderable  remainder  of  them,  it 
takes  a  chronic  course  through  many  years,  relapses  occurring  chiefly  in  the 
"tetany  season."  In  this  form,  trophic  disturbances  are  comparatively  fre- 
quent, nevertheless  its  prognosis  as  to  life  is  favorable,  since  no  case  of  death 
from  idiopathic,  uncomplicated  tetany  has  so  far  been  observed.  From  a 
diagnostic  point  of  view,  with  regard  to  the  "spasmophilia"  of  infancy  as  well 
as  to  the  tetanoid  conditions  of  adults,  reference  must  be  made  to  the  fact 
that  galvanic  overirritability  is  the  single  constant  and  obligatory  symptom 
of  tetany,  around  which  then  the  remaining  symptoms  can  group  themselves 
in  different  combinations.  In  a  child  in  a  condition  of  latent  tetany,  an  inter- 
current digestive  disturbance  or  taking  cold  can  at  any  time  lead  to  an  out- 
break of  spasm  in  the  extremities  or  to  spasm  of  the  glottis. 

Arthrogryposis,  an  affection  of  the  first  years  of  life,  which  is  charac- 
terized by  tonic  spasms  and  positions  of  contracture  of  one  or  several  limbs, 
lasting  from  days  to  weeks,  seems  to  have  relationships  with  infantile  tetany ; 
the  legs  are  fixed  now  in  extension,  again  in  flexion,  the  arms  spasmodically 
flexed.  Recovery  usually  occurs ;  Strwmpell,  however,  has  seen  two  cases  end 
fatally  (with  negative  post-mortem  findings).  Important  for  its  distinction 
from  true  tetany,  is  the  absence  of  mechanical  and  galvanic  irritability  of  the 
nerves. 

The  therapy  of  tetany  must,  of  course,  be  a  causal  one  in  all  secondary 
forms ;  so,  for  example,  in  lactation  tetany,  nursing  must  be  forbidden ;  in 
gastric  tetany,  according  to  conditions,  lavage  is  to  be  practiced  or — and  not 
too  late — pyloroplasty  or  gastroenterostomy  is  to  be  recommended,  etc.  In 
the  tetany  of  workers,  in  order  to  prevent  recurrence,  too  great  crowding 
together  of  these  people  is  to  be  prevented,  and  they  are  to  be  protected 
from  the  influence  of  cold;  further,  alcohol  is  to  be  forbidden  them;  eventually 
change  of  residence  and  calling  should  be  insisted  upon.  Parathyroid  tetany 
ought  to  be  dying  out  on  account  of  the  increasing  care  on  the  part  of  sur- 
geons to  avoid  injuring  the  parathyroids  in  goiter  operations. 

For  all  varieties  of  tetany  rest  in  bed  and  tepid  baths  of  long  duration 
are  to  be  recommended.  The  diet  should  be  purely  ovolacto-vegetarian  (with- 
drawal of  meat,  in  animals  experimentally  made  subject  to  tetany,  reduces  the 
frequency  and  intensity  af  the  spasms).  Of  drugs,  the  alkaline  bromides  (in 
daily  doses  of  from  3  to  6  grms — gr.  xlv-ojss.)  come  specially  under  consid- 
eration in  adults;  as  does  scopolamin,  0.0005  (gr.  1/120),  twice  a  day;  this 
last  acting  specially  on  the  pains,  for  which  also  chloral  hydrate,  salicylic 
preparations,  antipyrin,  pyramidon,  lactophenin,  etc.,  may  be  tried;  occa- 
sionally morphine  is  necessary.  Loeb  and  McCallum  recommend  calcium  lac- 
tate, which  reduces  the  overirritability  of  the  muscles  in  animals  without  para- 
thyroids.     A  teaspoonful   of  a   ten-per-cent.   solution   is   given   three   or   four 


THE    DYSKINESIAS  79 

times  a  day.  The  great  hopes  which  had  been  placed  upon  organotherapy 
with  parathyroid  preparations  have  not  been  realized.  The  transplantation 
of  parathyroid  tissues  also  has  given  no  result  worth  speaking  of,  since  these 
do  not  remain  capable  of  functioning  in  their  new  location,  but  speedily 
perish,  although  the  transplanted  structures,  so  long  as  they  are  not  reab- 
sorbed, act  favorably.  In  the  treatment  of  infantile  tetany,  on  account  of 
its  frequent  relation  to  rickets,  along  with  bromide  of  potassium  (see  formula, 
page  68)  and  chloral  (0.15  to  0.5 — grs.  2  to  7 — by  clysma),  phosphorus,  best 
given  in  the  form  of  Kassowitz's  emulsion,  plays,  with  right,  a  considerable 
role. 

I?      Phosphori     0.1    (gr.  V/2) 

01.    amygdal.    dulc 10.0   (oijss.) 

Sacch.  alb., 

Gunimi  mimosa   aa     5.0   (gr.  lxxv) 

Aq.  dest.,  q.  s. ;  ad  emuls 100.0    (§iij) 

M.     S.     A  teaspoonful  once  or  twice  a  day. 


LECTURE   V 
The  Dyskinesias 

D.     The  Choreiform  Diseases 

Involuntary,  quick  movements,  co-ordinated,  indeed,  but  nevertheless  of 
aimless  and  contrary  character,  which  are  not  rhythmic  but  rather  give  the 
impression  of  a  jerking,  continuous  unrest  of  the  affected  extremities,  we 
designate  chorea;  the  name  comes  from  the  Greek  (X"l"">  =  dance),  and  is  in 
so  far  suitable,  since  when  the  lower  extremities  are  affected,  the  gait  of  the 
patient  can  degenerate  into  dancing  and  hopping.  The  most  frequent,  and 
practically  by  far  the  most  important,  form  of  chorea  is  the  disease  known 
as  Chorea  minor,  Chorea  sancti  viti  ("Yeitstanz,"  "Danse  de  Saint  Guy"), 
or  Sydenham's  chorea;  much  more  rare  is  Huntington's  degenerative  or  hered- 
itary chorea,  which  is  also  an  autonomous  disease.  Usually  of  symptomatic 
indication,  on  the  other  hand,  are  hysterical  chorea  (also  "chorea  major"), 
which  occurs  auto-suggestively  by  imitation  of  chorea  minor,  and  not  infre- 
quently leads  to  school  or  class  epidemics,  chorea  in  lesions  of  the  cerebellar 
peduncles  (see  Lecture  XX),  as  well  as  pre-  and  post-hemiplegic  chorea,  which 
sometimes  is  observed  as  the  precursor  or  result  of  an  apoplectic  attack 
from  cerebral  hemorrhage. 

This  last  variety  of  chorea,  affecting  only  the  extremities  of  one  side,  is 
also  a  hemichorca.  Its  presence  justifies  the  conclusion  that  there  is  a  hemor- 
rhage into  the  optic  thalamus,  the  lenticular  nucleus,  or  into  the  most  pos- 
terior part  of  the  internal  capsule  of  the  opposite  side  of  the  brain.  The 
affected  extremities  show  lively  jerking  or  shaking  movements  ("hemiballism"), 
which  are  absent  during  sleep,  but  cannot  be  controlled  voluntarily,  even  may 
be  exaggerated  upon  attempting  this.  The  designation  of  certain  clinical 
pictures  described  as  great  rarities  by  the  name  "Electric  Chorea"  is  incor- 
rect. In  these  cases  the  involuntary  movements  follow  one  another  with 
lightning-like  rapidity,  in  the  form  of  violent  jerking.  Henoch's  form  of 
electric  chorea  is  probably  an  atypical  myoclonia,  that  of  Bergeron,  a  hys- 
terical phenomenon.  On  the  other  hand,  Dubini's  electric  chorea,  a  disease 
observed  in  Lombardy,  in  which  besides  muscular  twitchings  there  is  fever, 
pain  in  the  neck  and  back,  epileptiform  attacks,  loss  of  faradic  muscular 
irritability,  pareses,  etc.,  and  which  usually  terminates  fatally,  is  probably 
an  infectious  disease  of  obscure  etiology. 

1.     CHOREA    MINOR,    SYDENHAM'S    CHOREA 

This  disease,  in  the  great  majority  of  cases,  attacks  children,  chiefly  girls, 
particularly  between  the  sixth  year  of  life  and  puberty.  Younger  children 
are  only  exceptionally  attacked,  also  the  disease  is  very  rare  between  fifteen 

80 


THE    DYSKINESIAS  81 

and  twenty-five  years  (these  "juvenile  forms"  nearly  always  occur  in  females). 
Cases  occurring  at  a  later  age  are  great  rarities,  though  even  "senile  forms" 
have  been  known. 

In  the  etiology  neuropathic  predisposition  probably  plays  a  considerable 
role  (heredity  is  here  and  there  recognized).  In  the  majority  of  cases,  bow- 
ever,  the  action  of  some  infectious  influence  is  decisive.  In  the  first  place  must 
be  mentioned  acute  rheumatism  and  endocarditis,  which  often  precede  the 
attack  of  chorea  minor,  accompany  or  follow  it.  Less  frequently  a  connec- 
tion with  the  acute  exanthemata,  typhoid  fever,  pneumonia  or  erysipelas  has 
been  determined.  Exposure  to  cold  has  often  been  accused;  chorea  minor, 
indeed,  is  more  frequent  in  cold,  moist  localities  and  in  the  winter  months. 
In  the  juvenile  forms  pregnancy  always  plays  an  important  role.  It  is  seen 
in  young  primiparae,  who  are  attacked  in  the  early  months  of  pregnancy. 
The  disease  but  rarely  begins  suddenly.  As  a  rule,  the  family  notices  first  a 
general  unrest  with  psychical  disturbance  (anxiety,  ill  humor,  unsociableness), 
as  well  as  the  appearance  of  illness  and  poor  appetite;  in  this  stage  also 
many  patients  complain  of  weakness  and  vague  painful  sensations  in  the 
limbs.  Next,  movements  become  clumsy  and  finally  plainly  jerky;  there  is 
also  involuntary  grimacing.  Once  the  disease  picture  has  reached  full  devel- 
opment, the  choreic  character  of  the  movements  is  unmistakable;  we  notice 
a  continual  gesticulating,  there  is  no  longer  any  quiet  sitting  or  standing, 
short,  motiveless  movements  occur,  now  in  the  hands  and  fingers,  again  in 
the  feet,  now  an  arm,  now  the  head,  and  again  a  leg  is  jerked  hither  or  thither; 
a  wrinkling  of  the  forehead,  a  smacking  with  the  tongue,  winking,  protruding 
the  lips,  shows  involvement  of  the  muscles  innervated  by  the  cranial  nerves. 
The  French  speak  very  appropriately  of  a  "Folie  musculaire"  ("muscular 
madness").  In  walking,  the  patient  sometimes  gives  the  impression  of  a 
"jumping  jack.*'  which  moves  when  the  string  is  pulled.  In  severe  eases. 
however,  walking  is  impossible,  and  the  patient,  condemned  to  keep  her  bed. 
throws  herself  about  in  alarming  fashion.  The  muscles  of  the  diaphragm,  the 
oesophagus  and  the  larynx  may  be  involved  (irregular  breathing,  disturbances 
in  swallowing,  broken  speech)  even  in  the  pupils,  alternating  contraction  of 
sphincter  and  dilator,  a  so-called  "hippus,"  independent  of  the  influence  of 
light,  accommodation  or  convergence,  has  been  observed. 

Chorea  almost  always  begins  in  the  upper  limbs  and  in  the  face,  a  Heeling 
the  legs  later.  Not  infrequently  the  limbs  of  one  side  are  attacked  first;  as 
a  rule,  however,  this  original  "hemichorca"  sooner  or  later  passes  over  to 
the   other   side   also. 

Psychical  excitement,  even.,  the  consciousness  of  being  observed,  increases 
the  choreic  phenomena,  which,  however,  cease  during  sleep-  only  in  the  Very 
severe,  fatal  cases  the  intensity  of  the  movements  does  not  permit  sleep, 
and  the  patient   perishes  from  exhaustion   ("Status  ehoreicus"  ). 

In  chorea  minor  the  psyche  scarcely  ever  remains  entirely  intact;  how- 
ever, its  anomalies  are  usually  inconsiderable;  irritability,  a  high  degree  of 
dist ractibility,  an  ill-humored  deportment,  lability  of  mood.  In  the  above- 
mentioned  malignant  eases,  fortunately  rare,  on  the  contrary  there  may  he 
hallucinatory  delirium,   and   even   maniacal   attacks. 


82  LECTURE    V 

The  rare  symptoms  of  chorea  are:  slight  rise  of  temperature  (only  in 
status  choreicus  and  in  complications  with  polyarthritis,  endocarditis  acuta, 
etc.,  is  there  high  fever),  motor  weakness  ('"Chorea  mollis,"  incorrectly  "Chorea 
paralytica"),  pain  along  the  spinal  column.  On  account  of  the  anemia  often 
developing  with  chorea  minor,  sometimes  anemic  heart  murmurs  occur,  which 
should  not  he  confused  with  those  of  complicating  endocarditis.  On  tapping 
the  patellar  tendon,  it  is  sometimes  noticed  that  the  foot,  which  is  drawn  up 
by  the  contraction  of  the  quadriceps,  does  not  sink  down  again  immediately, 
hut  remains  raised  a  few  seconds  before  falling  (''tonic  reflex,"  Gordon's 
symptom). 

In  general  the  prognosis  is  favorable,  and  recovery  occurs  with  gradual 
lessening  of  the  symptoms  from  one  to  three  months  after  the  beginning  of  the 
disease.  However,  there  is  often  a  tendency  to  relapses  (these  occur  in  about 
25  per  cent,  of  the  cases).  Less  favorable  in  prognosis  are  the  choreas  oc- 
curring after  the  acute  exanthemata  and  typhoid  and  those  in  adults  in  which 
the  danger  of  endocarditis  is  very  great.  Most  dangerous,  however,  is  un- 
doubtedly chorea  gravidarum  with  a  mortality  of  30  per  cent. 

As  to  the  pathological  anatomy  of  Sydenham's  chorea,  the  lesions  found  in 
the  corpus  striatum  and  in  the  optic  thalamus  by  some  investigators  are  of 
interest.  Occasionally  multiple  emboli  have  been  found  in  these  parts,  to 
which  our  attention  had  been  already  directed  on  account  of  symptomatic 
hemichorea,  in  cerebral  hemorrhages  (Broadhurst)  ;  more  frequently  the  so- 
called  "chorea  bodies"  (Elischer,  Jakoteenko),  concentric  stratified,  highlv  re- 
fractile  bodies  are  seen  in  the  vascular  sheaths.  However,  all  these  findings 
are  still  the  subject  of  controversy. 

The  therapy  of  chorea  minor,  when  carefully  carried  out,  usually  gives 
very  satisfactory  results,  and  hence  will  be  considered  at  length.  On  account 
of  the  great  preponderance  of  the  disease  in  late  childhood  and  about  puberty, 
I  will  give  the  doses  of  medicine  for  children  of  from  ten  to  twelve  years; 
from  these,  those  suitable  for  younger  and  older  patients  can  easily  be  cal- 
culated. 

Every  case,  even  the  mildest  chorea  minor,  is  during  the  whole  duration 
of  the  disease  to  be  prevented  from  going  to  school,  to  public  nurseries,  etc., 
also  at  home  or  in  the  hospital  as  strict  isolation  as  possible  should  be  en- 
forced. The  patient  should  sleep  in  a  single  room,  should  not  eat  or  play 
with  the  other  children,  and  in  the  most  severe  cases,  apart  from  the  physician, 
should  see  only  one  and  the  same  nurse.  As  a  rule,  the  children  can  be  allowed 
to  amuse  themselves  with  picture  books,  quiet  games,  dolls,  etc.;  also  by 
unexciting  reading  (best  read  for  them).  Everything,  however,  in  small  "por- 
tions" only,  with  the  interposition  of  long  periods  of  complete  rest.  A  full 
measure  of  rest  in  bed  is  to  be  prescribed,  even  in  very  mild  cases  ;  for  example, 
from  8  p.m.  until  10  a.m.,  and  from  2  till  4  p.m..  namely,  sixteen  hours  in  bed. 
In  severe  cases  we  order  strict  rest  in  bed  until  there  is  decided  improvement ; 
in  very  severe  ones,  besides  this,  the  sick-room  is  kept  dark,  and  by  suitable 
padding  care  is  taken  that  the  patient  does  not  bruise  or  injure  herself.  The 
diet  should  be  purely  ovolacto-vcgetarian ;  alcohol,  coffee,  tea,  spices,  and  very 
salt    food   should  be   forbidden.      To   prevent   constipation   we  give   corn   bread 


THE    DYSKINESIAS  83 

and  plenty  of  fruit;  further,  taking  as  much  milk  as  possible  is  of  use;  for 
example,  a  half  cup  every  hour,  eventually  yoghurt,  kefir,  curds,  etc.  Where 
pure  milk  proves  unpleasantly  laxative,  the  addition  of  about  a  teaspoonful 
of  the  purest  powdered  gum  arabic  to  the  quart  is  to  be  recommended  (mix 
first  with  some  cold  milk,  add  the  rest  of  the  milk  and  boil  the  whole).  Daily 
tepid  baths  of  about  twenty  minutes'  duration,  followed  by  rubbing  down  with 
water  at  from  22°  to  25°  C,  act  favorably  also.  On  the  other  hand,  you 
are  expressly  warned  against  cold  douches  and  similar  applications.  From 
among  the  large  number  of  drugs  which  have  been  introduced  in  the  therapy 
of  Sydenham's  chorea,  only  three  appear  to  me  to  have  a  specific  effect:  ar- 
senic, antipyrin  and  cannabis  indica.  Arsenic  is  incontestable  the  most  effi- 
cient of  the  pharmacological  agents,  and  should  hence  be  preferred.  In  using 
it,  it  is  desirable  to  begin  with  small  doses  after  well-known  principles,  and 
gradually  to  increase  them  to  large  and  very  large  doses.  Chorea  patients 
are  especially  tolerant  of  arsenic,  and  it  almost  never  has  to  be  withdrawn 
on  account  of  digestive  disturbances,  herpes,  or  conjunctivitis.  To  antipyrin 
also  great  activity  is  attributed,  especially  by  French  pediatrists.  Neverthe- 
less, whA  it  is  the  only  drug  given  it  is  necessary  to  exhibit  it  in  such  large 
doses  (3.0-6.0  grm.  a  day  in  children  from  6  to  10  years  old,  5.0-6.0  grms. 
in  those  from  10  to  15  years),  that  we  must  reject  this  method  as  too  dan- 
gerous. The  matter  is  otherwise  in  its  combination  with  arsenic  preparations 
in  which  small  and  safe  doses  of  antipyrin  may  prove  a  good  adjuvant  to 
the  chief  drug.  A  combination  of  arsenic  acid  and  extract  of  cannabis  indica 
(provided  a  good  and  fresh  extract  is  used)  gives  even  better  results.  The 
"Pilulae  cannabinae  composita?,"  which  I  have  recommended,  have  proved  very 
useful. 

I£      Extract,  cannabis  indica; 0.3  (gr.  41/>) 

Acidi  arsenios 0.04-0.12  (0.15)  (gr.  %  to  1 ,'.-,) 

Quinin  sulphat 1.0  (gr.  xv) 

Extract,  valerian q.  s. 

Ut.  f.  pil.  No.  NXX. 

M.  S. — One  pill  three  times  a  day  after  meals  (daily  dose  of  extract  can- 
nabis  ind.,  0.03,   and   acid,   arsenic,  0.004-0.012,  or  eventually   0.015). 

Oilier  prescriptions  are: 

II      Scil.  Fowleri   .t gtts.  xxx-1 

A(|.  menth.  pip 80.0 

Sr.  simp] 20.0 

M.  S.—  A  dessertspoonful  in  water  three  linns  a  day  after  meals. 

K      Sol.  Fowleri 10.0 

Spts.   melissse  co 30.0 

M.  S.      Ten  (hops  in  milk,  from  one  to  three  times  a  day. 


84  LECTURE    V 

I?      Sodii  arseniatis 0.01-0.03  (gr.  %-V2) 

Antipyrin 5.0  (gr.  lxxv) 

Syr.  aurantii  cort 50.0   (§jss.) 

Aq.  dest q.  s.  ad    150.0   (%v) 

M.  S. 

A  teaspoonful  after  meals.  Daily  dose,  gr.  1/60  to  gr.  1/20  sodium 
arseniate,  and  71/>  grs.  antipyrin. 

Besides  these  drugs,  it  may  be  necessary  to  prescribe  hypnotics  for  a 
longer  or  shorter  time,  as  it  is  of  great  importance  that  the  patients  sleep 
long  and  deeply;  it  should  naturally  be  endeavored  to  get  along  with  the 
most  harmless  hypnotics  possible  and  in  order  to  prevent  habituation,  to  change 
them  often,  using,  for  example,  adalin,  bromural,  veronal  and  trional.  Occa- 
sionally an  evening  dose  of  2.0  grms.  of  bromide  of  potassium  will  be  sufficient ; 
salicylic  preparations  are  only  indicated  in  choreas  with  rheumatic  and  cardiac 
complications.  I  recommend  to  you  further  (but  only  after  the  irritative 
symptoms  of  chorea  are  plainly  on  the  decline)  to  let  the  patient  practice 
slow,  rhythmical  movements  of  the  extremities  upon  command,  best  while  lying 
in  bed.  Even  in  the  early  stages  as  well  as  at  the  height  of  the  disease,  however, 
the  undertaking  of  rational  breathing  exercises  is  to  be  recommended,  since 
many  chorea  patients  breathe  badly  and  superficially.  To  this  end  I  place  a 
pillow  under  the  sacral  region  of  the  patient  lying  on  his  back,  and  have  him 
practice,  for  five  to  seven  minutes  at  a  time,  deep,  rhythmical  inspiration  and 
expiration,  this  once  or  twice  a  day.  Whenever  it  is  possible  after  the  cure,  the 
patient  should  spend  from  four  to  six  weeks  at  some  quiet  resort.  In  anemic 
or  delicate  children  a  combination  of  this  with  salt  baths  or  a  course  of  iron  is 
advantageous. 

Chorea  gravidarum  can  furnish  a  cause  for  artificial  abortion ;  Sarvay 
gives  the  following  indications  for  this:  Prevention  of  the  taking  of  nourish- 
ment by  the  violence  of  the  irritative  phenomena,  loss  of  strength,  decided 
alteration   of  the   psychical   functions. 

2.  HEREDITARY  CHOREA,  HUNTINGTON'S  CHOREA 

In  1882  the  American  physician,  Huntington,  on  Long  Island,  called  atten- 
tion to  a  rare  affection,  which  characterized  in  the  main  by  progressive  chorei- 
form muscle  unrest  and  dementia,  attacks  chiefly  individuals  from  35  to  40 
years  old,  and  is  usually  marked  by  decided  heredity.  Sporadic  cases,  in- 
deed, sometimes  occur,  also  it  may  begin  between  25  and  30  or  between  40  and 
55  years  of  age,  while  only  very  exceptionally  do  the  initial  symptoms  commence 
about  puberty  or  even  earlier.  The  disease  has  the  tendency  to  begin  at  an 
earlier  average  age  in  each  succeeding  generation  ("anticipatory  heredity"). 
Still  less  frequently  than  in  America,  the  disease  occurs  in  the  Old  World, 
particularly  in  England  and  Germany.  As  exciting  causes,  psychic  traumata, 
exposure  to  cold,  the  puerperium,  excesses,  have  been  brought  forward.  Syphi- 
lis and  the  acute  infectious  diseases,  on  the  other  hand,  appear  to  play  no  role 
at  all.     Like  chorea  minor,  the  affection  usually  begins  in  the  arms  and  in  the 


THE    DYSKINESIAS  85 

face,  later  to  attack  the  legs;  also  it  is  sometimes  at  the  start  a  hemichorea.  In 
contradistinction  to  Sydenham's  chorea,  however,  patients  with  Huntington's 
disease  when  in  a  state  of  complete  quiet  and  abstraction  can  suppress  the 
shaking  in  carrying  out  intentional  movements  so  that  they  can  pursue  their 
occupations.  In  sleep  there  is  usually  complete  quiet  of  the  muscles;  psychi- 
cally a  depression  is  evident  which  cannot  be  said  to  be  motiveless,  since  the 
patients  are  only  too  well  aware  of  the  incurable  nature  of  the  family  scourge 
which  is  coming  upon  them  and  of  the  mental  failure  which  is  approaching;  in 
this  stage  suicide  or  attempts  at  this  are  common.  Later  the  patient  becomes 
apathetic  with  episodal  conditions  of  excitement,  the  memory  fails,  sometimes 
there  are  delusions  of  grandeur  or  of  persecution,  also  periods  of  hallucinatory 
confusion,  mental  failure  continually  advances,  and  there  can  be  finally  com- 
plete dementia.  There  are,  however,  cases  with  relatively  little  disturbance 
of  intelligence,  and  there  is  even  a  variety  of  the  disease  beginning  at  puberty, 
which  usually  becomes  stationary  after  some  time,  and  does  not  alter  the 
mental  personality.  Apart  from  this  last  form,  the  prognosis  is  very  un- 
favorable; the  disease  can,  indeed,  last  for  twenty  or  more  years,  so  that 
the  patients  not  infrequently  live  to  be  sixty  or  seventy  years  old;  death 
ensues  in  dementia  and  marasmus,  or  from  intercurrent  diseases. 

In  its  pathologico-anatomical  relations  we  have  a  number  of  heterogeneous 
findings  whose  interpretation  is  not  yet  possible.  The  newest  investigations 
are  those  of  Alzheimer.  He  finds  very  grave  changes  in  the  cerebral  cortex, 
as  well  as  in  the  corpus  striatum  and  in  the  nucleus  ruber,  namely,  a  sur- 
rounding and  in  part  a  destruction  of  the  ganglion  cells  by  the  "amoeboid" 
elements  of  the  glia. 

The  therapy  is  purely  symptomatic,  and,  as  such,  gives  little  prospect  of 
results.  Only  in  very  early  stages  arsenic  and  scopolamin  (V->-2  nag.  per  dose 
once  a  day)  appear  to  be  able  to  procure  alleviation.  It  may  be  attempted 
to  modifv  somewhat  the  choreiform  unrest  by  sedative  hydriatic  procedures, 
curative  exercises,  etc.  It  is  best  to  bring  these  patients  as  soon  as  possible 
into  the  seclusion  of  an  institution  where,  apart  from  the  elimination  of  all 
irritants,  intelligent  care  of  the  episodal  states  of  excitement  (baths,  opium, 
veronal,  rest  in  bed,  etc.)  can  best  be  carried  out.  In  the  latest  stages  the 
care  of  the  disturbed  and  demented  patient  is  particularly  difficult. 

E.     The  Athetoses 

In  the  year  1871  attention  was  called  by  the  American  neurologist  II  a  m- 
monil  to  a  peculiar  phenomenon  of  motor  irritation  which  he  designated  by 
the  name  Athetosis  (afleTOff  =  restless).  The  hands  and  feet,  that  is,  the 
individual  fingers  and  toes  of  the  patients,  make  constant,  slow  excursions^ 
beginning  with  hyperextension  (analogous  to  the  movements  id'  the  tentacles 
of  an  octopus),  which  do  not  cease  during  sleep,  hut  up  to  a  certain  point 
can  he  controlled  by  an  effort  of  the  will.  Fig.  .'{1  shows  one  phase  of  athetoid 
movements  of  the  hand  after  an   instantaneous  photograph.     It   refers  to  a 

child  in  whom  the  athetosis  developed  as  a  result  of  an  acute  encephalitis. 
In  fact,  focal  lesions  which  are  situated  in  the  parts  of  the  thalamus,  some 


86 


LECTURE    V 


times  also  in  those  of  the  lenticular  nucleus,  bordering  on  the  internal  capsule 
(more  rarely  also  in  the  internal  capsule  itself,  that  is,  in  the  posterior  third 
of  its  posterior  limb),  can  produce  not  only  hemichorea,  as  already  mentioned, 
but  also  hemiathetosis,  on  the  opposite  side.  The  last,  indeed,  is  much  more 
frequent  than  cerebral  hemichorea.  Children  are  particularly  predisposed. 
There  are  indeed  transition  forms  between  hemichorea  and  hemiathetosis ;  also 
in  lesions  of  the  basal  ganglia  on  both  sides  there  is  a  bilateral  athetosis. 

Besides  this   symptomatic  athetosis   there  is   also   an  idiopathic   form,  the 
so-called  "double  athetosis,"  a  disease  in  itself  without  a  certainly  determined 
anatomical  substratum,  in  which  the  motor  phenomena  described  present  the 
only  pathological  manifestation,  which  usually  is  present  from  birth  or  develops 
in  earliest    childhood.      Here,  besides  the  extremi- 
ties,  the   muscles    of   the    face   and   neck   are   also 
affected ;     the     patients     continually     distort     the 
mouth,  make  faces,  constantly  turn  and  twist  the 
head,  and,  on  account  of  athetosis  of  the  tongue 
muscles,  are  much  embarrassed  in  speaking.     The 
condition  is  a  stationary  one,  although  remissions 
occur.     Whether  their  occurrence  is  to  any  extent 
favored  by  drugs,  as  arsenic,  bromides,  cannabis, 
or    scopolamin,    or    by    hydriatic    procedures,    is, 
however,  a  question. 

Related  to  double  athetosis,  probably,  is  a  rare 
progressive  and  incurable  disease,  that  Oppcnhcim 
has  described  as  "Dysbasia  lordotica  progressiva," 
Ziehen  as  "torsion  neurosis."      It  affects  children 
between  eight  and  fourteen  years  old  (mainly  Rus- 
sian Jews).      The  affection  is  characterized  by   a 
lordoscoliosis    of  the   lower   portion   of   the   verte- 
bral column,  evident  only  on  walking  and  standing, 
usually    disappearing    when    the    patient    is    lying 
down,  along  with  flexion  and  external  or  internal 
rotation  of  the  legs.     In  the  rotators  of  the  thigh,  also,  in  the  tibialis  anticus 
and   in  the  biceps,  along  with  tonic  tension,  clonic   contractions   may   occur. 
The  electrical  irritability  is  unaltered. 


Athetosis  (instantaneous  photo- 
graph). 


F.     Paralysis  Agitans 

By  this  name  James  Parkinson,  in  1817,  denominated  a  disease  charac- 
terized by  muscular  rigidity  and  peculiar  shaking  movements,  which  in  honor 
of  its  discoverer  has  also  been  called  Parkinson's  disease.  Paralysis  agitans 
appears  in  the  vast  majority  of  cases  between  the  fortieth  and  the  sixtieth 
years  of  life,  and  in  general  affects  by  preference  the  male  sex;  many  adthors 
assert  that  the  poorer  classes  of  the  population  are  more  frequently  attacked. 
Unexplained  conditions  of  local  nature  appear  here  to  exert  an  influence; 
since  in  our  neighborhood,  where  the  disease  is  relatively  frequent,  there  is 
no  evidence  of  a  less  predisposition  to  it  in  the  higher  classes  of  society.     Its 


THE    DYSKINESIAS  87 

etiology  is  quite  obscure;  mental  stress,  physical  overexertion,  exposure  to 
cold  and  trauma,  have  been  accused;  it  is  probable  that  these  factors  may 
occasionally  play  the  role  of  exciting  causes  (namely,  when  the  first  symp- 
toms of  the  disease  appear  directly  in  an  overstrained  or  traumatized  limb), 
but  in  a  majority  of  cases  there  is  nothing  of  the  sort,  and  we  must  confess 
our  lack  of  knowledge  with  regard  to  the  real  causal  connection.  The  hered- 
itary occurrence  of  the  disease  has  been  only  noted  as  a  great  rarity. 

Parkinson's-  disease  usually  begins  gradually  and  unremarked;  the  patient 
notices  a  slight  general  fatiguability,  little  by  little  a  temporary  tremor,  which 
is  at  first  limited  to  one  hand  (oftener  the  right),  but  gradually  becomes  a 
permanent  symptom,  and  finally  attacks  the  remaining  extremities  one  after 
another.  At  the  same  time  a  continual  tension  of  the  muscles  which  slows  all 
movements  is  added  to  the  tremor;  in  a  minority  of  the  cases  the  last  phe- 
nomenon  precedes   the   appearance  of  the  tremor. 

In   the   fully  developed  disease  the  tremor  presents  the   following  charac- 


Fig.  32. 
Paralysis  Agitans.     Handwriting. 

tiers:  it  is  rhythmical,  slow  (two  to  five  oscillations  a  second),  persists  during 
complete  rest,  to  cease  during  skip,  and  in  the  hands  imitates  certain  com- 
plicated movements,  namely,  "pill  rolling"  and  "coin  counting."  On  carrying 
out  a  movement  the  tremor  usually  lessens,  and  indeed,  when  the  disease  is 
not  far  advanced,  may  cease,  so  that,  for  instance,  a  hunter,  on  aiming,  can 
keep  still  and  make  the  shot.  Less  frequently  the  tremor  increases  somewhat 
upon  movement.  Excitement  always  increases  the  shaking.  While  at  the 
start,  writing,  eating,  dressing,  etc.,  are  still  possible,  in  the  advanced  stages 
(tin-  disease  can  last  twenty  years  or  more)  these  functions  are  impossible. 
The  tremor  of  the  legs  makes  itsc] f  evident  bv  the  patient,  when  seated,  "beat- 
ing time"  with  his  foot;  Usually  the  oscillations  in  the  upper  and  lower  ex- 
tremities are  synchronous. 

The  attitude  of  Parkinson  patients  is,  as  a  rule,  uncommonly  typical  (see 
Fig.  •'!•■>,.  The  hiad  and  trunk  are  held  forward,  arms  and  legs  addueted. 
knees  and  elbows  flexed,  in  the  hands  the  metacarpophalangeal  joints  flexed, 
the    middle    and    end     phalanges    extended,     the    finger-tips     of    the     thumb    and 

index  finger  held  together.  This  attitude  is  maintained  so  firmly  that  all  the 
joints  mighl  be  thougl     I  >  be  contractured.     (Only  exceptionally  do  the  pa- 


LECTURE    V 


tients  stand  straight  upright  in  statuesque  stiffness — Charcot's  "extension 
type").  The  features  have  the  immobility  of  an'  antique  mask,  only  the 
eyes  move  in  a  lively  manner;  in  looking,  turning  of  the  head  is  avoided,  the 
patient  turns  himself  rather,  as  a  whole,  about  his  axis.  This  occurs  slowly 
with  the  aid  of  a  number  of  small  steps ;  especially  all  locomotion  is  usually 
begun  with  very  slow,  deliberate  steps,  so  that 
the  gait,  indeed,  has  something  solemn  about  it; 
little  by  little,  however,  the  steps  often  become 
more  rapid,  "tripping,"  the  bent  forward  pa- 
tient "runs  after  his  center  of  gravity,"  in  order 
not  to  fall,  and  finally,  in  order  to  stop  his  for- 
ward movement,  has  to  bring  up  against  the 
wall  or  some  other  object.  This  we  call  "pro- 
pulsion." Analogous  phenomena  are  "retro- 
pulsion"  and  "latero-pulsion,"  which  we  make 
evident  by  giving  the  patient  a  slight  push  back- 
ward or  sideways.  Parkinson  patients,  on  ac- 
count of  the  hypcrtonus  of  all  their  muscles, 
as  well  as  on  account  of  the  fact  that  they  can 
only  carry  out  an  intentional  movement  with 
more  or  less  delay,  no  longer  have  the  power 
of  regaining  the  balance  of  their  bodies  which 
have  been  put  out  of  equilibrium. 

Disturbances  manifest  themselves  also  in  the 
speech  muscles ;  the  voice  is  weak  and  plaintive, 
the  speech  slow,  sometimes  chopped  off,  remind- 
ing one  of  that  of  a  rider  upon  a  horse  which 
is  trotting  fast.  In  two  of  my  patients  I  ob- 
served a  continual  chattering  of  the  teeth. 

Sensibility  is  for  objective  tests  practically 
always  normal ;  exceptionally  there  is  slight  re- 
duction of  sensation  in  the  hands  and  feet.  On  the  other  hand  many  patients 
complain  of  drawing  sensations  in  the  extremities,  of  cramplike  pains  in  the 
calves,  of  paresthesia  in  the  face  ("as  if  something  was  crawling  on  it"),  prin- 
cipally, however,  of  a  burning  heat  of  the  surface  of  the  bod}*,  particularly 
upon  the  abdomen  and  upon  the  back.  On  this  account  patients  with  paralysis 
agitans  gladly  uncover  themselves  while  in  bed. 

The  reflexes  are  usually  exaggerated,  less  frequently  normal.  The  electric 
muscular  irritability  is  unaltered.  The  "paradoxal  foot  phenomenon"  of  West- 
phal  is  often  found;  if  one  brings  the  foot  of  the  patient  into  a  position  of 
extension  (dorsal  flexion)  the  tibialis  anticus,  extensor  digitorum,  and  peronei 
contract,  since  their  origin  and  their  insertion  are  brought  nearer  together, 
so  that  the  foot  remains  for  a  long  time  in  the  position  given  it. 

In  most  cases  there  is  often  a  very  distressing  salivation.  Hyperidrosis 
is  rarer.  A  not  very  infrequent  variety  of  paralysis  agitans  is  Parkinson's 
disease  without  agitation.  In  this,  with  otherwise  typical  symptomatology, 
the  characteristic  tremor  is  absent. 


Typical  Attitude  in  Paralysis 
Agitans. 


THE    DYSKINESIAS  89 

The  prognosis  of  the  disease  is  not  unfavorable  as  to  life,  but  very  un- 
favorable as  to  recover}' ;  remissions  occur,  but  unfortunately  they  are  not 
usually  of  long  duration.  In  the  late  stages  the  fate  of  the  unhappy  invalid 
is  a  wretched  one,  since  with  fully  retained  intelligence  he  is  condemned  to 
absolute  helplessness,  and  must  depend  upon  outside  aid  for  the  most  ele- 
mentary needs  (for  example,  for  turning  himself  in  bed,  wiping  off  the  saliva 
that  flows  from  his  mouth).  Death  follows  either  in  marasmus  or  from  inter- 
current diseases,  among  which  cerebral  hemorrhage  is   relatively   frequent. 

What  is  the  anatomical  basis  of  this  terrible  disease?  As  to  this  question 
it  was  formerly  classed  among  the  "functional  neuroses."  To-day,  however, 
it  is  known  that  in  the  spinal  cord  in  Parkinson's  disease,  proliferation  of  the 
glia  and  abnormal  pigmentation  of  the  cells  of  the  anterior  horn,  together 
with  changes  in  the  vessels,  are  to  be  found  (Redlieh,  Sander,  Dubief,  Ballet), 
also  the  muscles  show  changes  according  to  some  authors  (Blocq,  Gautliier) . 
Nevertheless,  it  is  not  far-fetched  to  consider  these  not  very  characteristic 
lesions,  which  may  be  interpreted  as  an  accentuation  of  senile  changes,  as  only 
secondary,  and  to  think  of  another  "primum  movens,"  perhaps  in  the  glandular 
apparatus  of  our  bodies.  So  Roussy  and  Clunet  would  make  the  parathyroid 
glands  responsible,  claiming  to  have  found  in  them  changes  which  they  desig- 
nate by  the  expression  "Hyperparathyroidosis."  According  to  Haberfeld, 
indeed,  the  changes  found  are  due  only  to  senility.     The  question  is  still  open. 

With  regard  to  the  treatment  of  Parkinson's  disease,  Oppenhcim's  remark 
is  recommended  for  your  consideration:  "The  physician  can  in  this  disease 
do  much  harm  and  little  good."  In  order  to  avoid  the  first,  one  should  refrain 
from  trying  cold  water  treatment,  exercises,  sun-baths,  vigorous  massage, 
faradization,  while — indeed,  seldom  enough — carrying  out  careful  passive 
movements,  giving  tepid  baths  (or  the  cooler,  indifferent  warm  baths,  and  even 
electric  baths)  and  a  mild  vibration  massage  can  alleviate  the  symptoms.  More 
useful  as  palliatives  are  certain  drugs,  above  everything  hydrobromide  of 
Bcopolamin,  either  in  the  form  of  injections  or  as  tablets.  Of  this,  from 
().()()()•_'  to  0.0004  (1/300  to  1  1.50  gr.)  once  or  twice  a  day:  also  duboisin  sul- 
phate  in  the  same  dose,  usually  moderates  the  tremor  and  muscular  tension  con- 
siderably. I  have  given  both  medicines,  sometimes  alone  and  sometimes  com- 
bined, for  years,  without  having  observed  any  symptoms  of  intoxication.  One 
must  not  decide  upon  this  continuous  method  of  administration  too  soon,  since 
it  is  a  last  refuge.  As  long  as  the  disease  is  not  at  a  too  advanced  stage  it  is 
well  to  give  this  medicine  from  time  to  time  for  a  period  of  twenty  days;  in 
the  interim  an  arsenic  cure  is  to  be  recommended;  for  example,  Sol.  Fowleri, 
in  doses  of  two  drops,  increasing  from  twice  to  seven  times  a  day.  and  then 
slowly  returning  to  twice  a  day;  also  the  drinking  of  arsenic  containing  waters 
[Dwrkhevmer-Mawquelle,  Val  Sinestra,  Levico)  can  be  carried  out  after  the 
manner  recommended  at  these  resorts.  Of  other  drugs  recommended  instead 
of  Bcopolamin  and  duboisin,  from  my  own  experience  I  can  mention  as  occa- 
sional! v  efficient,  tinct.  veratri  viridis  (two  or  three  times  a  day,  8-4  drops  in 
thin  mucilage).  To  be  strictly  avoided  are  all  drugs  increasing  sweating, 
namely,  the  salicylic  preparations.  Most  patients  who  have  been  given  para- 
thyroid substance  experimentally  have  reacted  with  an  increase  of  their  trouble 


90  LECTURE    V 

(I  could  convince  myself  of  this  in  one  case),  which  would  support  the  views 
of  Roussy  and  Clunet.  Placing  the  patient  among  as  quiet  surroundings  as 
possible,  preventing  all  exciting  visits,  pleasures,  etc.,  are  important ;  still 
more  important  in  the  later  stages  is  the  choice  of  a  nurse  of  inexhaustible 
patience  and  great  skill  in  carrying  out  all  the  technique  of  the  difficult  and 
tiresome  nursing. 

G.     The  Myotonias 

A  peculiar  disease  condition  was  made  known  by  the  Schleswig  physician 
Thomsen  in  1876.  His  material  was  furnished  by  his  own  family,  which  in 
five  generations  had  presented  over  twenty  cases.  This  "Thomsen's  dis- 
ease" later  received  the  name  of  Myotonia  congenita.  In  the  vast  majority 
of  cases  it  is  observed  as  a  hereditary  family  complaint ;  sometimes  consan- 
guinity of  the  parents  is  to  be  recognized;  very  frequently  in  the  relatives  a 
heaping  up  of  the  most  varied  psychoses  and  neuropathies  are  found.  As 
exciting  causes  acting  upon  the  latent  predisposition,  physical  excesses  play 
the  greatest  role;  rarely  fright  or  trauma,  very  rarely  infectious  diseases, 
are  accused. 

The  disease  is  either  first  noticed  in  early  childhood,  or  it  begins  about  the 
time  of  puberty,  or  somewhat  later  (for  example,  at  the  time  of  entering  the 
army).  Ninety  per  cent,  of  the  cases  are  in  males.  In  general  the  symptoms 
of  the  disease  increase  slowly  and  continually  during  a  number  of  years,  then 
become  stationary;  later,  indeed,  the  intensity  of  the  symptoms  may  again 
decrease,  though  such  remissions  are  not  very  frequent ;  a  recovery,  on  the 
other  hand,  is  excluded.  The  disturbance  pathognomonic  of  Thomson's  disease 
is  characterized  by  the  persistence  of  a  condition  of  muscular  contraction 
opposed  to  the  intended  end  on  the  carrying  out  of  a  voluntary  movement. 
If  the  patient,  after  long  periods  of  rest,  attempts  to  carry  out  any  movement, 
this  follows  in  the  promptest  manner ;  the  groups  of  muscles  brought  into 
contraction  together  can,  in  spite  of  every  effort  of  the  will,  be  relaxed  again 
only  after  from  5  to  30  seconds.  For  example,  if  we  ask  the  patient  to  shake 
hands,  he  cannot  let  go  for  some  time.  If,  however,  the  patient  undertakes 
the  movement  a  second,  third,  or  fourth  time,  we  notice  that  the  inhibition 
of  relaxation  ceases  each  time  after  a  shorter  interval,  so  that  finally  the 
movement  can  take  its  normal  course.  If  these  patients  march,  at  first  they 
stand  as  if  rooted  to  the  ground,  then'  follow  the  first  most  difficult  steps 
which  are  interrupted  by  repeated  tonic  contractions.  Little  by  little,  however, 
"the  machine  gets  going,"  and  finally  long  distances  are  traversed  without  any 
trouble;  the  patient  can  even  dance.  All  voluntary  muscles  can  be  the  seat 
of  the  myotonic  disturbances  of  movement.  However,  as  a  rule,  the  muscles  of 
respiration  are  free,  those  of  the  trunk  and  neck  less  involved  than  those 
of  the  limbs,  and  of  these  last  the  upper  less  than  the  lower.  The  muscles  of 
the  face,  of  mastication,  the  external  eye  muscles,  the  tongue,  and  even 
the  muscles  of  the  pharynx  and  larynx,  can  be  affected;  while  the  myotonic 
dyskinesia  makes  itself  apparent  on  grimacing  and  putting  out  the  tongue 
in  most  cases;  in  phonation,  turning  the  eyes,  swallowing  and  chewing,  it   is 


THE    DYSKINESIAS  91 

seldom  observed.  Psychical  excitement,  cold  and  dampness  increase  the  myo- 
tonic symptoms. 

Almost  always  there  is  increase  in  volume  of  the  muscles,  on  account  of 
which  the  patients,  in  spite  of  usually  somewhat  subnormal  strength,  present 
an  athletic  appearance. 

The  tendon  reflexes  are  either  normal,  they  exhibit  tonic  contractions,  or 
they  are  reduced ;  they  are  seldom  absent.  The  mechanical  and  electric  mus- 
cular irritability  are,  as  Erb  has  shown,  very  characteristically  altered.  Per- 
cussion of  the  muscles  sets  up  the  formation  either  of  a  depression  or  of  a 
swelling  which  does  not  disappear  for  from  5  to  30  seconds.  Direct  faradic 
stimulation  of  muscles  shows  increased  irritability;  on  stimulating  with  strong 
currents  persistent  myotonus  appears,  on  continued  strong  faradization  there 
is  sometimes  a  decided  "muscle  wave."  Direct  galvanic  irritation  brings  out 
the  most  striking  anomalies;  slow  and  persistent  character  of  the  contractions, 
abnormally  low  threshold  of  irritation,  loss  of  the  contractions  on  opening  the 
circuit,  AnCIC  =  or  >KC1C;  besides  this,  sometimes  upon  the  stabile  appli- 
cation of  strong  currents  there  are  rhythmical  undulations  from  the  cathode 
to  the  anode  (sometimes  only  appearing  after  repeated  application  of  the 
current).     These  different  anomalies  Erb  sums  up  as  the  "Myotonic  reaction." 

As  a  pathological  change  a  uniform  hypertrophy  of  all  the  fibers  depending 
upon  an  increase  of  the  undifferentiated  protoplasm  is  found,  while  the  con- 
tractile substance  and  its  morphological  expression  the  striation  is  defective. 
The  sarcolemma  shows  increase  of  nuclei,  the  fibrilli  vacuolization.  Besides 
this  there  is  slight  increase  of  the  interstitial  connective  tissue.  Knoblauch 
thinks  that  there  is  an  abnormal  preponderance  of  the  red,  slow-acting  muscle 
fibers  as  compared  to  the  light,  quick-acting  ones,  and  that  there  is  a  hyper- 
trophy of  the  first.  Along  with  these  muscle  changes,  opposing  relations  of 
innervation  can  play  a  role,  as  Jaqnct  has  shown  by  myographic  investigations. 

From  a  therapeutic  point  of  view,  above  everything  else  prevention  of  the 
factors,  which  are  shown  by  experience  to  work  unfavorably,  is  important 
(  keeping  the  patient  warm,  prevention  of  emotional  excitement,  suitable  occupa- 
tion). Further,  systematic  exercises,  warm  baths  and  massage  can  alleviate 
the  troubles.  The  drugs  here  and  there  recommended  (strychnine,  antipyrin, 
testicular  and  thyroid  extract,  atropin,  iodide  of  potassium),  since  they  are 
useless,  have  been  entirely  given  up.  A  moderate  amount  of  alcohol,  on  the 
contrary,  acts  favorably.  The  formerly  practiced  nerve  stretching,  which  has 
u  its  object  causing  decrease  of  the  muscular  hypertrophy  by  injuring  the 
nerves,    is    to   be   warned   against. 

Varieties  of  myotonia  are:  I'.  Myotonia  atrophica,  a  combination  of  Thom- 
sin's  disease  with  progressive  muscular  atrophy,  to  be  described  in  the  nexi 
lecture  ;   this   is  not    at    all    rare. 

-.  Myotonia  acquisita  {Talma,  Jolly),  an   incurable  condition  character 

Bed  by  the  myotonic  reaction  which  follows  infectious  diseases  and  traumata, 
in  which,  however,  there  is  usually  a  certain  muscular  rigidity  even  when  al 
ii^l,  while  long-continued  movement   favors  the  occurrence  of  myotonus. 

.'5.  Paramyotonia  congenita  (Eulenberg),  \<vy  rare.  This  is  a  condition 
of  permanent  muscular  contraction  which  usually,  under  the  influence  of  cold, 


92  LECTURE    V 

occurs  in  certain  muscle  groups  (of  the  neck,  face,  muscles  of  deglutition,  and 
those  of  the  extremities)  and  relaxes  in  from  a  quarter  of  an  hour  to  several 
hours.  The  myotonic  reaction  is  absent,  the  histo-pathological  picture,  how- 
ever, much  resembles  that  in  Thomscns  disease.  The  disease  is,  as  a  rule, 
decidedly  a  hereditary  family  one,  and  is  observed  immediately  after  birth. 

H.     Congenital  Muscular  Atony 

Oppenheim,  in  the  year  1900,  first  called  attention  to  a  congenital  path- 
ological condition  which  he  denominated  "Myatonia  congenita."  This  name, 
however,  has  the  disadvantage  that  it  furnishes  an  opportunity  for  confusion 
with  the  myotonia  congenita  described  above.  On  this  account  I  consider  the 
expression  "Congenital  muscular  atony"  more  correct.  In  such  children  im- 
mediately after  birth  a  striking  lack  of  movement  makes  itself  evident.  This 
is  chiefly  limited  to  the  legs ;  in  severe  cases,  however,  it  can  also  affect  the  arms, 
the  thorax,  the  neck,  so  that  the  patient  gives  the  impression  of  complete 
paralysis.  Nevertheless,  it  is  only  pseudo-paralysis ;  by  painful  stimuli  weak 
movements  of  protection  can  be  set  up.  The  muscles  are  doughy,  but  not 
atrophied.  The  muscular  flaccidity  is  so  extreme  that  one,  for  example,  can. 
raise  the  shoulders  up  above  the  ears,  can  bring  the  feet  up  behind  the  neck 
and  cross  them  there,  etc.  In  severe  cases  the  limbs  hang  like  loose  appendages 
to  the  body.  The  tendon  reflexes  are  absent,  the  skin  reflexes  usually  re- 
tained. Electric  irritability  of  the  muscles  to  both  varieties  of  current  is, 
as  a  rule,  reduced,  seldom  lost ;  reaction  of  degeneration  is  never  present ; 
other  anomalies  cannot  be  made  out.  The  disease  is  never  progressive ;  on 
the  other  hand,  it  shows  a  tendency  very  slowly  to  improve.  Recovery  is 
probably  the  most  frequent  termination ;  we  endeavor  to  accelerate  it  by 
strong,  deep-acting  faradization.  After  an  electro-therapeutic  treatment  a 
temporary  reappearance  of  the  patellar  reflex  is  sometimes  to  be  noted,  more 
frequently  the  appearance  of  a  few  voluntary  movements,  which  then  become 
more  and  more  a  permanent  acquisition.  Further,  irritants  favoring  move- 
ment, and  later  exercises,  come  into  consideration ;  finally,  carefully  managed 
strychnine  and  arsenic  cures.  The  prognosis  is  impaired  by  susceptibility  to 
fatal  broncho-pneumonias  in  case  the  respiratory  muscles  are  attacked.  The 
disease  is  not  hereditary,  almost  never  a  family  one.  A  unique  anatomical 
substratum  does  not  appear  to  be  present.  In  one  case  examined  by  me,  a 
piece  of  muscle,  removed  for  diagnosis,  showed  only  abnormal  richness  in 
nuclei.  Other  observers  have,  however,  seen  loss  of  the  cross  striation  and_ 
striking  irregularities  in  the  contour  and  caliber  of  the  fibers.  Also  anomalies 
have  been  found  in  the  spinal  cord,  for  example,  abnormal  smallness  of  the 
motor  cells  of  the  anterior  horn. 

A  clinical  likeness  to  congenital  muscular  atony  is  presented  by  the  "myo- 
pathia rachitica,"  isolated  by  Hagenboch  and  myself  between  190-i  and  1907, 
a  muscular  disease  never  congenital,  but  developing  in  rachitic  children,  in 
which  "snake  man  like"  positions,  crossing  the  feet  behind  the  neck,  etc.,  are 
found  exactly  as  in  the  former.  Histological  alterations  are  found  in  this 
disease   in   relatively   few  and  severe   cases,   and   are   then   characterized,   as  I 


THE    DYSKINESIAS 


93 


have  shown,  by  uniform  disturbance  of  growth  of  the  muscular  tissue  in  the 
direction  of  a  regression  to  a  comparatively  undifferentiated  stage  (see  Fig.  3-i). 


I.    Periodic  or  Paroxysmal  Paralysis 

This  disease,  whose  first  description  (1882)  comes  from  the  Russian  physi- 
cian Schachnowicz,  very  often,  but  not  always,  occurs  as  a  family  disease,  and 
usually  in  early  life  The  affected  persons  present  at  intervals  of  different 
length,  transitory  paralyses  of  extensive  muscular  groups.  These  rarely  affect 
only  the  legs  after  the  paraplegic  type,  usually  the  arms  are  also  attacked, 
often  the  muscles  of  the  trunk,  too,  while  the  muscles  of  the  face  and  the  eyes 
almost  always  remain  unaffected.  The  par- 
alysis, usually  most  intense  in  the  legs  and 
starting  here,  as  a  rule,  can  be  complete, 
or  only  moderate  paresis.  On  electric  exam- 
ination direct  and  indirect  irritability  is  re- 
duced at  the  beginning  of  the  attack  and 
while  it  lasts.  The  paralysis  is  usually 
flaccid:  lure  and  there,  however,  increased 
tension  of  individual  muscles  has  been  ob- 
served; the  tendon  reflexes  in  the  paralyzed 
parts  are  reduced  or  lost,  only  occasionally 
exaggerated;  the  skin  reflexes,  on  the  other 
hand,  are  normal.  The  paralysis  is  little  by 
little  established  in  the  course  of  about  an 
hour ;  movement  of  the  body  delays  its  ap- 
pearance ;  on  this  account  nocturnal  attacks 
are  especially  frequent.  Return  to  normal 
is  accomplished  by  degrees  after  a  few  hours; 
the  paroxysms,  however,  repeat  themselves 
at  intervals  of  different  lengths  (days, 
(reeks,  or  even  months).  Between  the  in- 
dividual attacks  the  patients  are  quite  healthy.  It  is  probably  a  disease  due 
to  autointoxication;  the  abnormally  constituted  muscles  appear  to  react  to 
the  formation  of  a  poison  which  is  accumulated  during  rest,  by  failure  of 
function:  the  urine  voided  during  the  attack  shows  increased  toxicity,  often 
contains  acetone.  Therapeutically,  massage  and.  strong  faradization  are  rec- 
ommended; further,  atropin  in  daily  doses  of  0.002  until  mild  intoxication  is 
beginning;  bromine  and  iodine-salts,  strychnine  and  eserine  have  been  tried 
without  any  results  worth  speaking  of. 

In  this  connection  "paralyzing  vertigo"  (Girlicr) — "Kubisagari"  (Miura) 
may  be  mentioned.  It  is  a  disease  occurring  in  stable-men  in  the  canton  of 
Geneva,  and  endemic  in  Japan,  probably  of  miasmatic  origin,  and  recovering 
upon  removal  from  the  atmosphere  of  the  stable.  It  consists  also  in  flaccid 
periodically  occurring  paralyses,  with  intervals  of  health,  hut   it  begins  with 

very   severe   vertigo,   besides   the  extremities    it    attacks   also    tin-   muscles   of  the 

peck  and  the  levatores  palpebrarum,  and  can,  indeed,  avoid  the  muscles  of  the 


Fio.  34. 
Rachitic  Myopathy. 


94  LECTURE    V 

limbs.  Difficulty  in  swallowing,  paralysis  of  the  masseters,  diplopia,  and 
amblyopia  are  not  infrequent.  Not  rest,  but  work,  seems  to  favor  the  out- 
break of  the  attacks. 

J.    Myasthenia 

Myasthenia  gravis  pseudoparalytica,  or  the  Erb-Gohlflam  disease,  is  a 
disease  which  is  characterized  by  excessive  fatigability  of  the  muscles,  leading 
to  paralytic  weakness,  and  for  which  the  muscles  supplied  by  the  motor  cranial 
nerves  show  special  predilection. 

This  comparatively  rare  disease  usually  develops  gradually  (commonly 
between  the  ages  of  twenty  and  forty  years,  and  in  women  somewhat  more 
frequently  than  in  men).  As  to  its  etiology  we  know  nothing  certain ;  if  in 
the  anamnesis  we  hear  very  frequently  of  exhausting  diseases  having  been 
passed  through  or  of  severe  overexertion,  nevertheless  on  account  of  the  rarity 
of  the  disease  we  must  assume  that  these  factors  exert  their  pathogenic  action 
only  upon  the  ground  of  an  already  present  predisposition.  There  is  probably 
a  congenital  inferiority  of  the  muscular  system ;  in  this  sense  speaks  its  rela- 
tively frequent  accompaniment  by  malformations ;  for  example,  of  the  toes 
and  fingers,  of  the  mamma,  of  the  genitals,  and  of  the  lungs,  or  by  progressive 
muscular  dystrophy,  which  likewise  develops  upon  an  endogenic  basis.  (See 
the  next  lecture.) 

A  more  and  more  rapidly  appearing  fatigability  which  leads  to  more  and 
more  complete  paralysis  (which  disappears  on  rest)  makes  itself  felt,  first  in 
the  external  eye  muscles,  the  mimic  muscles,  those  of  the  pharynx,  the  masti- 
catory muscles,  and  those  of  the  tongue;  later  the  phenomenon  usually  extends 
also  to  the  territory  innervated  from  the  spinal  cord — neck,  trunk  and  ex- 
tremities. All  muscular  action  in  the  region  of  the  affected  structures  begins 
at  first  without  delay ;  after  a  few  movements,  however,  the  contraction  is  less 
extensive,  always  feebler  in  its  results,  and  finall}'  the  muscle  is  entirely  para- 
lyzed, or  better,  pseudo-paralyzed,  since  after  a  more  or  less  extended  rest,  its 
function  is  regained.  Least  manifest  is  this  anomaly  in  the  morning  after  awak- 
ening, increasing  during  the  day  and  reaching  its  maximum  by  evening.  Not 
all  muscles  are  equally  fatigable ;  most  severely  affected,  we  find  almost  al- 
ways, those  which  are  naturally  used  a  great  deal,  so,  above  all,  the  eye  muscles. 
Hence  double  vision  as  well  as  failure  of  the  levator  palpebrae  (ptosis)  belong 
to  the  most  invariable  symptoms  of  myasthenia.  Besides  this,  it  leads  to  facial 
diplegia,  to  disturbances  of  speech,  chewring  and  swallowing,  to  falling  of  the 
head  upon  the  breast,  to  exhaustion  paralysis  in  the  arms  and  legs  (namely 
at  the  root  of  the  extremities)  ;  occasionally,  however,  the  muscles  of  the 
trunk  and  limbs  remain  unaffected  while  their  involvement  alone  is  of  the  great- 
est rarity. 

The  disease  has  a  great  tendency  to  intermissions,  in  whicn  the  patients  for 
weeks,  months  and  years  can  be  entirely  free  from  trouble;  termination  in  re- 
covery is,  on  the  other  hand,  rare,  and  the  prognosis  is  on  this  account  un- 
favorable, since  the  harmless  initial  stage  of  the  disease  can  stretch  over  ex- 
ceedingly long  periods  of  time  characterized  by  long  intermissions  and  short 


THE    DYSKINESIAS  95 

exacerbations.  (In  one  case  of  Curshmann's,  twenty-two  years.)  Once  the 
myasthenia  has  reached  its  full  development,  however,  the  situation  is  exceed- 
ingly dangerous.  The  pseudo-paralysis  is  constantly  more  permanent,  the  mus- 
cles recover  almost  not  at  all.  Again  the  symptoms  are  more  severe  in  the 
external  eye  muscles  and  give  the  picture  of  external  ophthalmoplegia.  The 
voice  is  aphonic  on  account  of  failure  of  the  larynx  muscles;  the  face  re- 
sembles a  death  mask  ;  the  pseudo-paralysis  of  the  extremities  and  of  the  muscles 
of  the  back  and  neck  compels  the  patient  to  keep  his  bed ;  on  taking  food  he 
chokes,  and  fluid  nourishment  regurgitates  through  the  nose  if  it  does  not  enter 
the  trachea.  Conditions  favoring  the  occurrence  of  hypostatic  and  aspiration- 
pneumonia  are  hence  furnished  in  the  highest  degree ;  if  paralysis  of  the 
respiratory  muscles  (perhaps  also  of  the  heart)  does  not  sooner  put  an  end 
to  life. 

Clinical  examination  of  the  diseased  muscles  shows  with  entire  absence  of 
atrophy,  fibrillary  contraction  and  reaction  of  degeneration,  almost  always, 
the  so-called  "myasthenic  reaction"  which  can  be  defined  shortly  as  an  over- 
fatigability  for  the  faradic  current :  if  a  muscle  is  faradized  continually  for 
half  a  minute  to  one  minute,  a  uniform  reduction  in  the  contraction  is  ob- 
served, which  according  to  the  strength  of  the  stimulus  is  lost  entirely,  sooner 
or  later.  A  pause  of  scarcely  a  minute  suffices,  however,  to  allow  the  stimulus 
to  become  again  active  as  before.  The  faradic  tetanization  can  be  carried  out, 
not  continuously  hut  at  intervals  of  from  two  to  three  seconds ;  the  contrac- 
tion is  then  observed  to  become  weaker  at  each  stimulation  until  finally  the 
muscle  no  longer  responds,  to  react  again,  however,  when  it  has  been  per- 
mitted some  rest.  The  myasthenic  reaction  is  obtainable  from  the  nerve  as 
well  as  from  the  muscle;  it  is  not  absolutely  pathognomonic  of  the  Erb-Gohljium 
disease,  as  it  has  been  found  in  different  organic  nervous  diseases  and  in  a 
slight  form  even  in  healthy  people.  Overfatigability  of  the  tendon  reflexes  by 
repeatedly  eliciting  them  is  occasionally  to  be  found;  they  usually  remain  nor- 
mal, however. 

As  far  as  the  pathological  anatomy  of  the  disease  is  concerned  it  is  certain 
that  the  brain,  spinal  cord  and  peripheral  nerves  remain  normal  in  myasthenia. 
In  the  muscles  themselves,  however,  changes  have  been  repeatedly  found,  of 
which  the  mosl  uniform  is  the  increase  of  lymphoid  cells  figured  by  Weigert. 
Knoblauch  has  made  an  abnormal  preponderance  of  the  light  muscle  fibers  over 
the  dark  ones  responsible,  hence,  according  to  his  view,  the  Erb-Goldflam  dis- 
ease   is   the  opposite   dl'  ThoTmen's  disease   (sic   page  90).      The   fact    that 

here  and  then'  persistence  of  flic  thymus,  or  tumors  of  this  body  have  been 
found  at  autopsy  in  myasthenics,  attracts  attention  I"  its  possible  connection 
with  internal  secretions.  Lundborg,  on  theoretical  considerations,  has  con- 
ceived myasthenia  as  being  a  hyperparathyroidosis,  as  an  expression  of  the 
o\i  il'nnction  of  the  parathyroid  glands.  On  the  other  hand  I  have  not  been 
aide  to  find  the  least  trace  of  myasthenia  or  the  myasthenic  reaction  in  rats 
which  had  been  made  artificially  hyperparathyroiditic  by  Tselin  by  gland  trans- 
plantation. In  its  pathogenetic  relation  MarVMSCO  attributes  an  important 
role  to  an  insufficiency  in  format  ion  of  the  oxydase  necessary  for  the  recupera- 
tion  of   the   working  muscle. 


96  LECTURE    V 

The  treatment  of  myasthenia  gravis  pseudo-paralytica  must  above  every- 
thing be  a  therapy  of  avoidance.  No  electro-therapy !  Even  electro-diagnostic 
testing  is  not  to  be  undertaken  oftener  than  is  absolutely  necessary.  During 
the  exacerbations  of  the  disease  rest  in  bed;  during  the  intermissions,  long 
periods  of  entire  rest  introduced  into  the  regime  of  the  day.  Rest  cures  in  the 
open  air,  at  climatic  stations  where  possible,  should  be  carried  out  every  year. 
On  the  other  hand  all  athletic  exercises  are  to  be  strictly  forbidden.  Naturally 
the  disease  causes  unfitness  for  military  service.  Hydrotherapy  is  to  be  avoided. 
In  the  end  stage  the  use  of  the  oesophageal  tube  is  to  be  avoided  as  much  as 
possible,  since  Oppcnhcim  has  observed  sudden  death  from  asphyxia  while  it 
was  being  passed.  Long  periods  of  rest  after  meals  should  be  enforced.  Nour- 
ishment must  be  selected  according  to  the  viewpoint  of  the  most  nutrition  in 
the  smallest  volume.  Of  drugs,  the  combination  of  arsenic  and  strychnine  is 
to  be  recommended;  for  example,  Iy  Sol.  Fowleri,  5.0;  tinct.  nuc.  vom.,  10.0. 
M.  S—  6  to  12  drops  t.  i.  d. 


LECTURE    VI 

The  Progressive  Muscular  Atrophies 

Gentlemen  :  When  we  speak  to-day  of  "Progressive  Muscular  Atrophies," 
we  use  this  term  in  a  certain  sense  as  a  proper  name  for  quite  definite  chronic 
degenerative  processes  in  the  motor  apparatus.  In  no  way,  however,  for  other 
conditions  setting  in  with  progressive  degeneration  of  the  muscles.  Such  de- 
generation,  indeed,  can  occur  upon  the  basis  of  a  great  number  of  organic 
nervous  diseases,  of  which,  for  example,  we  have  already  become  acquainted 
with  the  polyneuritides,  and  in  later  lectures  will  study  amyotrophic  lateral 
sclerosis,  syringomyelia,  and  other  conditions.  We  differentiate  a  myopathic, 
a  neural  and  a  spinal  form  of  "progressive  muscular  atrophy"  whose  different 
anatomical  and  clinical  peculiarities  we  will  proceed  to  study.  At  the  start, 
however,  we  must  understand  that  many  facts  warn  us  against  drawing  the 
line  bit  ween  these  three  subvarietics  too  schematically.  On  the  one  hand  there 
are  numerous  clinical  transition  forms,  on  the  other,  autopsies  have  shown  that 
in  the  neural  form  almost  always,  in  the  myopathic  not  rarely,  changes  are 
found  in  the  spinal  cord. 

Common  to  the  whole  group  is  its  endogenic  basis,  which  in  the  myopathic 
and  neural  forms,  shows  itself  through  its  predominantly  hereditary  family 
occurrence,  while  in  the  spinal  form  a  more  frequent  nonfamily  type  stands  on 
the  one  hand,  a  rarer  family  type  on  the  other.  The  congenital  inferiority, 
upon  the  basis  of  which  the  progressive  muscular  atrophies  develop,  shows  it- 
silf  in  an  occasional  combination  with  other  heredo-degenerative  diseases  or 
with  malformations  and  congenital  defects.  (For  example,  with  myotonia  con- 
genita (see  page  90);  further,  with  hereditary  ataxia,  with  family  spastic 
paraplegia,  with  congenital  muscular  anomalies,  with  funnel  chest,  skull  and 
jaw  deformities,  atrophy  of  the  bones,  symmetrical  exostoses,  hemihyperplasia 
of  the  skeleton,  shortening  of  the  thumb,  idiocy  and  deafmutism.  etc.) 

A.     The  Myopathic  Form 

This  is  also  called  primary  progressive  muscular  atrophy;  much  used,  how- 
ever, in  German-speaking  countries,  is  the  name  proposed  by  Erb,  "Dystrophia 
Musculorum  Progressiva"  (Progressive  .Muscular  Dystrophy),  while  in  France 
Charcot  procured  the  adoption  of  the  term  "Myopathic  primitive  progressive." 

As  already  said,  this  is  a  disease  of  chiefly  hereditary-family  occurrence, 
which  nearly  always  begins  in  early  life  within  the  two  first  decades,  and  is  un- 
noted at  tin-  start.  The  first  Symptoms  of  muscular  wasting  make  themselves 
evident    at    the    roots    of   the   e\  t  remit  ies    and    Upon    the    trunk,    seldom    about     the 

head,  almost  never  in  the  peripheral  segments  <>f  the  limbs,  which,  as  a  rule, 
remain   intact   even    in    the  later  course   of   the  disease.      In   the  muscles   under- 

97 


98  LECTURE    VI 

going  atrophy,  reaction  of  degeneration  is  almost  never  observed,  but  only 
simple  quantitative  reduction  of  irritability.  Fibrillary  contractions  are  also 
absent.  The  usually  symmetrically  affected  muscles  show  a  particularly  char- 
acteristic peculiarity,  namely  that,  along  with  the  atrophic  processes  there 
occur  changes  which  cause  an  increase  in  volume  of  the  affected  parts  and 
striking  abnormalities  in  the  form  of  the  body;  the  combination  of  this  "pseudo- 
hypertrophy" of  certain  muscles  with  atrophy  of  others  is  comparatively  reg- 
ular; sensibility,  the  function  of  the  sense  organs,  the  sphincters  of  the  bladder 
and  of  the  rectum  remain  normal. 

Along  with  cases  impressing  themselves  by  typical  heredo-family  occur- 
rence in  which  transmission  occurs  chiefly  through  the  mother,  who  herself, 
however,  can  remain  free  from  the  disease — I  designate  this  last  as  "maternal 
metrapectic  inheritance,"* — come  also  sporadic  cases,  namely,  in  the  "juvenile 
scapulohumeral  type"  (Erb)  which  we  will  consider  more  at  length  under  the 
discussion  of  the  special  forms  of  dystrophy.  Not  infrequently  infectious 
diseases,  trauma,  or  overexertion  give  the  signal  for  the  outbreak  of  the  disease; 
the  same  factors  also  may  cause  new  progress  after  temporary  arrest. 

Pathological  J  natomy 

The  disease  process  which  lies  at  the  basis  of  this  trouble  has  been  thor- 
oughly studied  by  Erb  and  Marinesco.     Macroscopically  the  "fish  flesh"  ap- 


Fig.  35. 

Alteration  of  the  Muscle  in  Progressive  Muscular  Atrophy.     Hematoxylin-eosin-osmic  acid 

stain.    Gastrocnemius.    Zeiss  Oc.  2,  Obj.  E. 

pearance  of  the  affected  muscles,  which  are  plainly  distinguishable  from  the 
healthy  brown-red  muscles,  but  hardly  at  all  from  the  surrounding  fatty  tissue 
in  color,  is  particularly  striking.  In  sections  (see  Fig.  35)  the  striking  in- 
equality in  size  of  the  fibers  is  first  noticed.  While,  according  to  Erb,  the 
extremes  in  the  normal  cross   sections  of  the  fibers  vary  between  20  and  80 

*  A?rexc"'==To  avoid,      tt\  ht)tt)p  =  The  mother. 


THE    PROGRESSIVE    .MUSCULAR    ATROPHIES  99 

microns  and  ninety  per  cent,  of  the  fibers  measure  between  20  and  60  microns, 
among  a  larger  or  smaller  contingent  of  such  normal  sized  fibers  are  found 
numerous  atrophic  ones  measuring  from  7  to  15  microns,  also  hypertrophic 
ones  of  100,  125  even  200  microns  and  more.  The  hypertrophy  of  the  fibers 
seems  to  represent  the  first  stage  of  the  process,  the  atrophy  the  latter  one; 
it  finally  passes  over  to  entire  loss  of  fibers  when  only  empty  sarcolemma 
sheaths  are  left.  One  need  not  think  that  in  pseudo-hypertrophic  muscles  the 
thick,  and  in  macroscopically  atrophic  muscles,  the  thin,  fibers  especially  pre- 
dominate, since -nothing  is  more  like  the  parenchyma  of  an  atrophic  muscle 
in  dystrophy,  under  the  microscope,  than  the  tissue  of  a  pseudo-hypertrophic 
one.  Determining  the  total  volume,  indeed,  is  the  smaller  or  greater  degree 
of  proliferation  of  fat  and  connective  tissue.  Both  can  remain  within  moderate 
bounds  or  may  reach  an  excessive  degree. 

The  muscle  fibrilli  almost  all  show  a  considerable  increase  of  both  the  border 
and  of  the  interior  nuclei.  Many  are  fragmented,  split,  or  show  in  the  central 
part,  round  vacuoles  that  look  as  if  punched  out.  However  great  the  atrophy 
of  the  fibers,  the  striation  is  plainly  preserved  everywhere.  In  the  areas  most 
diseased  the  longitudinal  striation  usually  seen  is  increased  to  definite  fibrilla- 
tion; upon  longitudinal  sections  the  muscles  sometimes  appear  bordered  with 
rosary-like  swellings.  The  intramuscular  vessels  undergo  to  a  great  extent  en- 
doarteritic  and  mesoarteritic  changes  which  can  cause  great  thickening  of  their 
walls;  in  the  periarterial  connective  tissue,  and  also  in  the  perimysum,  consid- 
erable increase  of  nuclei  is  often  found;  this  can  increase  to  definite  areas  of 
infiltration.  In  contradistinction  to  these  constant  changes  in  the  muscular 
tissue,  the  lesions  in  the  anterior  horn  cells  of  the  spinal  cord,  found  in  only 
exceptional  cases,  fall  entirely  into  the  background.  Since,  however,  the 
trophic  centers  for  the  muscles  lie  in  these  last,  it  is  conceivable  that  functional 
alterations  of  these  cells  are  responsible  for  the  perverse  method  of  develop- 
ment and  disturbances  of  nutrition  in  the  muscles,  even  in  those  cases  in  which 
the  anterior  horn  cells  appear  morphologically  unaltered. 

CLINICAL    BEHAVIOR   OF   THE    DYSTROPHIC   MUSCLES 

Upon  investigating  the  muscles  of  dystrophic  patients  we  will  find  naturally 
different  conditions  depending  upon  whether  we  are  observing  an  atrophic  or  a 
pseudo-hypertrophic  region. 

In  the  first  case  the  muscles  arc  either  uniformly  thin  as  a  whole,  which 
process  can  go  so  far  that  the  affected  portions  of  the  extremities  look  like 
those  of  a  skeleton,  or  in  the  jniddle  of  the  muscle  among  the  atrophic  parts. 
round,  sometimes  globular  prominences,  are  found.  This  phenomenon,  which 
sometimes  is  first  seen  when  the  muscle  contracts,  is  entirely  pathognomonic  of 
muscular  dystrophy  and  appears  as  "partial  pseudo-hypertrophy"  to  present 
a  transition  to  the  second  manner  of  appearance,  dystrophic  myopathy.  In 
tliis  last  form  the  affected  muscles  increase  in  mass  to  such  an  extent  that  ath- 
letic contours  are  produced  (Fig.  86).  On  palpation  one  experiences  in  them 
either  a  firm,  often  peculiar  fluctuating  rubber-like  resistance,  or  (in  advanced 
stages)  on  the  other  hand,  a  peculiar  dead  feeling.     Here  and  there  I  have  seen 


100 


LECTURE    VI 


linear  atrophies  of  the  skin  like  the  well  known  striae  of  pregnancy  in  women, 
over  such  pseudo-hypertrophic  muscles.  It  can  happen  that  a  muscle  whose 
ability  to  functionate  is  almost  suspended  by  the  dystrophy,  presents  a  normal 
outline;  in  it  the  atrophic  and  the  pseudo-hypertrophic  process  balance  one 
another.  That  atrophic  or  pseudo-hypertrophic  muscles  shorten  and  produce 
contractures  like  that  in  Fig.  38,  as  well  as  that  visible  in  the  left  leg  of  the 
boy  in  Fig.  36,  is  not  a  frequent  occurrence. 

With  the  morphological  anomalies  related,  disturbances  of  function  go  hand 
in  hand ;  the  strength  and  the  extent  to  which  the  affected  muscles  are  able  to 


Fig.  36. 
Progressive  Muscular  Dystrophy.    Pseudo-hypertrophic  form. 


contract,  in  the  course  of  the  disease  continually  grow  less;  at  the  same  time 
the  electrical  and  mechanical  irritability  continually  decrease,  to  be  finally 
lost.  The  exceedingly  rare  cases,  in  which,  besides  this,  indications  of  reaction 
of  degeneration  are  found,  are  usually  otherwise  atypical  and  probably  should 
be  considered  as  transition  types  to  the  neural  and  spinal  forms  of  progressive 
muscular  atrophy. 

The  tendon  reflexes  in  the  affected  region  decrease  and  are  finally  lost. 

Progressive  muscular  dystrophy  has  its  "favorite  muscles."  Other  muscles 
it  attacks  only  exceptionally,  still  others  it  regularly  avoids.    As  to  this  peculiar 


THE  PROGRESSIVE  MUSCULAR  ATROPHIES 


101 


electivity,  you  may  be  guided  by  the  following  table  in  which  I  have  also 
indicated,  by  italicizing,  the  muscles  which  are  particularly  predisposed  to 
pseudo-hypertrophy. 


Usually    Affected. 

Pectoralis  major  (pars  ster- 

nalis) 
Pectoralis  minor 
Latissimus  dorsi 
Serratus  anticus  major 
Rhomboideus 
Trapezius     (especially     pars 

inferior) 
Erectores  spinae 
Deltoid 
Biceps  brachii 
Brachialis  antieus 
Supinator  longus 
Olutei 
Quadriceps 
Adductores  femoris 
Peronei 

Tibialis  anticus 
Gastrocnemius 
Orbicularis  oris 
Orbicularis  palpebrarum 


Less  Frequently 
Affected. 

Sternocleidomastoid 

Infraspinatus 

Supraspinatus 
Levator  scapula; 
Coracobraehialis 
Triceps 
Teres  major 

Teres  minor 
Abdominal  muscles 
Sartorius 
Tensor  fasciae  Iatae 


Exceptionally 
Affected. 

Diaphragm 

Masticatory   muscles 
Eye  muscles 
Heart  muscle 
Tongue  * 


TYPICAL    ANOMALIES    OF    CONFIGURATIONS    AND    ATTITUDE 

Looking  at  the  nude  bodies  of  dystrophic  patients  we  are  impressed  by  the 
fact  that  the  deep  depressions  of  the  atrophic  parts  and  the  great  swellings  of 
the  pseudo-hypertrophic  ones,  join  immediately  on  to  the  well- formed  portions 
of  the  limbs.  On  this  account  the  whole  figure  takes  on  an  inharmonious  out- 
line. Besides  this,  however,  we  come  face  to  face  with  a  number  of  exceedingly 
characteristic  local  disfigurements,  for  example,  the  picture  of  scapula-  alatse 
which  occupied  us  already  in  the  first  lecture;  this  occurs  on  account  of 
elimination  of  the  action  of  the  serrati  (see  Fig.  37).  If  to  this  is  added,  how- 
ever, that  of  the  pectorales,  the  trapezii  and  the  latissimus  dorsi,  the  shoulder- 
blades  with  the  attached  arms  become  loose  appendages  of  the  thorax,  and  we 
speak  of  "loose  shoulder."  The  atrophy  of  the  sternal  portion  of  the  pectoralis 
causes  a  flattening  of  the  thorax;  under  certain  circumstances,  indeed,  the 
sternum  and  ribs  form  a  sort  of  a  fissure.  Decided  lordosis  occurs  on  account 
of  the  fact  that  extension  at  the  hip-joint  is  impaired  and  the  center  of  gravity 
must  be  moved  forward;  tin'  weakening  of  the  contractions  of  the  abdominal 
muscles  can  increase  this  disfigurement  to  the  highest  degree;  between  the 
approximated  shoulder-blades  and  the  hips,  a  number  of  cross  folds  produced 
by  the  forcing  together  of  the  soft  parts  are  observed.  The  "gnomes  calves" 
occur  on  account. of  the  pseudo-hypertrophy  of  the  gastrocnemii,  the  "tapir 
snout"  through  the  trunk  like  increase  of  volume  of  the  upper  lip.  Through 
imperfections  of  the  orbicular  muscles  of  the  eves  and  the  mouth  the 
"myopathic  fades"  is  produced,  the  gloomy  rigid  "Sphinx  countenance."     If 

•Our  patient  of  Fig.  36  showed  {.Teat  enlargement  of  the  tongue.     Besides  this  nn  enlarge- 
ment of  the  heart  was  demonstrable  by  percussion  and  by  orthodiagraphy. 


102  LECTURE    VI 

such  a  patient  laughs,  however,  on  account  of  the  imperfect  function  of  the 
orbicularis  oris,  his  mouth  is  drawn  into  an  ugly  fissure  ("transverse  laugh- 
ing") while  the  two  nasolabial  folds  impose  themselves  ps  deep  vertical  furrows, 
the  "coups  de  hache"  of  the  French;  the  atrophy  of  the  trunk  muscles  can  lead 
to  the  so-called  "wasp  waist." 

INDIVIDUAL  FORMS  OF  PROGRESSIVE  MUSCULAR  DVSTROPHY 

We   have   passed   before   us   the    characteristic   anomalies    which    fully   de- 
veloped cases  of  dystrophy  present.      We  must  now,    however,  go     into    the 


Fig.  37. 
Progressive  Muscular  Dystrophy.     ('type  "Erb.")     Winged  Scapula. 

definite  grouping  of  symptoms  which,  characterized  also  by  peculiarities  as  to 
the  time  of  beginning  and  course,  impose  themselves  as  special  types  of 
dystrophy.     We  distinguish: 

1.  Forms  with  initial  and  preponderant  involvement  of  the  pelvic  girdle 
and  thigh  muscles. 

a.  Atrophic  variety  (type,  Leyden-Moebius) . 

b.  Pseudo-hypertrophic  variety  (type,  Duchennc-Gr'u  singer). 

2.  Forms    with    initial    and    preponderant    involvement    of    the    shoulder- 
girdle  and  upper  arm  muscles. 

a.  Juvenile,  scapulo-humeral  variety  (type,  Erb). 

b.  Infantile,  facio-scapulo-humeral  variety  (type,  Landouzif-Dejerlne). 
The  pelvic-femoral  forms  (la  and  lb)  begin,  as  a  rule,  in  childhood  and 

affect  by  preference  boys.  The  atrophic  variety  usually  begins  later  than  the 
pseudo-hypertrophic;  the  first  usually  in  the  8th  to  10th  year  of  life,  the  latter 
about    the   3d    to    5th   year.      The   first    symptom    which    usually    strikes    the 


THE    PROGRESSIVE    MUSCULAR    ATROPHIES 


103 


parents  is  a  waddling  gait  caused  by  weakening  of  the  gluta'i.  The  children 
fall  easily  and  experience  great  difficulty  in  climbing  stairs.  Often,  on  walk- 
ing, they  let  the  feet  fall  stamping,  a  result  of  the  quadriceps  paralysis.  A 
lordosis  is  usually  early  apparent  also.  If  we  lay  such  a  child  upon  the  floor 
and  then  let  him  rise,  on  account  of  the  weakness  of  his  back,  thigh,  and  leg 
extensors,  he  is  compelled  first  to  assume  the  position  of  a  quadruped  and 
then,  by  aid  of  his  hands,  to  climb  up  his  legs,  as  it  were.  In  the  pseudo-hyper- 
trophic  Duchenne-Griesinger  type,  the  great  increase  in  volume  of  the  calves 
and  buttocks  makes  itself  early  evident  (Fig.  36)  ;  while  in  time  the  disease 
also  involves  the  shoulder-girdle  and  upper  arm  muscles,  pseudo-hypertrophy 


Fig.  38. 

Excessive  Clubbing  of  the  Foot  on  Account  of  Contracture  of  the  Gastrocnemius  in  Progressive 
Muscular  Atrophy. 


usually  dots  not  occur  in  these;  later,  indeed,  it  falls  off  in  the  glutei  and 
gastrocnemii  to  give  place  to  atrophy.  The  muscles  of  Hie  face  are  later  and 
less   regularly  affected   in  the  hypertrophic  variety  than  in   the  atrophic. 

Of  the  two  shoulder-girdle  upper  arm  types,  that  of  Erb,  the  "juvenile" 
(2,  a,  sec  Fig.  37)  begins  in  youth  (or  even  during  the  third  decade  of  life) 
and  usually  develops  \rv\-  slowly,  while  the  Landouzy-Dejerine,  or  "infantile" 
variety,  commences  in  early  childhood  and  spreads  somewhat  more  rapidly. 
In  tin-  muscles  of  Hie  shoulder-girdle  and  upper  arm  mentioned  in  the  table 
on  page  101,  in  I  lie  infantile  type  at  the  same  time  in  those  of  the  face,  an 
increasing  weakness  and  atrophy  become  apparent.  In  ErVs  type  pseudo- 
hypertrophy is  common  (in  the  deltoid,  triceps  and  infraspinatus),  in  the 
Landouzy-Dejerine  type  il   is  almost  always  absent.     Only  when   the  last  has 

later  attacked   ihe   legs   also,   it    may   cause    increase   in    volume   of   the  calves,   like 

Erb' s  t  vpe. 

After  we  have  exposed  the  four  chief  forms  of  progressive  muscular 
dystrophy  in  their  chief  differential  points,  we  will  not  neglect  to  point  out 
how  very  frequently  atypical  cases  come  under  observation;  whether  it  is, 
that  the  time  and  the  topography  of  the  commencement  of  the  diseases  do  not 


104  LECTURE    VI 

agree  with  one  another  (for  example,  when  a  pelvic  and  thigh  form  does  not 
begin  until  the  end  of  the  twentieth  year  or  even  later),  or  that  the  grouping 
of  the  affected  muscles,  or  the  order  of  their  involvent  presents  unusual 
relations. 

Differential  Diagnosis 

All  in  all,  one  can  say  that  progressive  muscular  dystrophy  in  its  fully 
developed  stage  can  hardly  be  confused  with  any  other  disease;  as  to  its 
special  differences  from  the  "spinal"  and  the  "neural"  forms  of  progressive 
muscular  atrophy,  I  will  refer  in  this  connection  to  a  description  of  these  last. 
In  the  early  stages,  on  the  other  hand,  one  must  be  on  his  guard  against 
diagnostic  errors.  The  abnormal  manner  of  rising  occurs  in  beginning 
spondylitis,  in  circumscribed  acute  lumbar  polyomyelitis,  in  abnormally 
localized  post  diphtheritic  paralysis,  in  rickets;  the  waddling  gait  in  rickets 
and  osteomalacia;  the  "gnome-like"  calves  in  spastic  spinal  paralysis  and  in 
hydrocephalus;  myotonia  congenita  runs  its  course  with  increase  in  volume  of 
the  muscles,  this  can  occur  locally,  also,  as  a  congestive  phenomenon  in  throm- 
bosis of  veins ;  finally,  there  is  a  genuine  congenital  muscular  hypertrophy. 
Tlje  opposite  to  the  last  condition  is  furnished  by  congenital  muscle  defects 
which,  indeed,  attack  by  preference  the  "favorite  muscles"  of  dystrophy  (Erb, 
Bing,  etc.),  but  are  almost  always  asymmetrical. 

Course  and  Prognosis 

Though  pauses  for  ten,  fifteen,  and  even  thirty  years  may  interrupt  the 
progress  of  muscular  dystrophy,  nevertheless  its  course  is  almost  always 
steadily,  though  slowly,  progressive.  In  general  it  is  the  rule  that  in  the 
average  the  cases  beginning  early  and  the  pelvic-femoral  forms  run  the  most 
rapid  course.  Such  patients,  as  a  rule,  die  comparatively  young,  since  the 
earlier  they  become  permanently  bedridden,  the  more  threateningly  hangs  over 
them  the  Damocles'  sword  of  tuberculosis  and  broncho-pneumonia,  to  which 
most  of  them  fall  victims.  Patients  with  the  juvenile  type  of  the  disease,  how- 
ever, may  reach  the  age  of  seventy  years  or  more.  That  progressive  muscular 
dystrophy  is  the  direct  cause  of  death  is  exceedingly  rare ;  in  such  cases  the 
result  depends  upon  the  involvement  of  the  diaphragm  and  respiratory 
muscles ;  still  rarer,  however,  are  recoveries  of  typically  dystrophic  children, 
as  they  have  been  observed  by  Erb  and  Marina. 

Treatment 

What  can,  and  shall  we  do,  not  to  put  a  stop  to  this  fatal  disease  (since 
this  does  not  lie  within  our  power),  but  at  least  to  oppose  its  progress  as  much 
as  possible?  In  the  first  place  it  is  necessary  to  improve  the  general  condition 
as  much  as  possible  by  good  air  and  by  nourishing  food,  not  too  rich  in  fats 
and  carbohydrates.  Care  should  be  taken  that  daily  such  an  amount  of  exer- 
cise and  movement  as  his  condition  permits,  without  the  production  of  a 
feeling  of  fatigue,  should  be  carried  out  by  the  patient.     "Ne  quid  nimis" 


THE    PROGRESSIVE    MUSCULAR    ATROPHIES  105 

should  also  be  the  motto  in  treatment  by  massage  and  electricity ;  the  last  I 
apply  only  in  the  form  of  the  "electric  hand"  with  very  weak  faradism,  or  as 
galvanization,  and  I  would  warn  you  directly  against  producing  faradic  con- 
tractions of  the  muscles.  I  have  here  and  there  seen  good  results  from  salt 
baths  beginning  with  very  weak  concentrations  and  increasing  gradually  to 
moderately  strong  ones,  also  from  prolonged  tepid  baths  and  corresponding 
indifferent  thermal  ones,  that  is,  a  decided  improvement  in  the  function  of  the 
muscles.  As  regards  attempts  at  medication,  however,  I  can  only  report 
failures  from  the  much  used  strychnine  and  from  thyroid  and  hypophysis 
preparations;  also  the  results  of  injections  of  muscle  juice  vaunted  by 
certain  enthusiasts  have,  as  was  to  be  expected,  remained  unconfirmed.  Not 
infrequently  we  are  in  a  position  to  call  in  the  aid  of  the  orthopedist;  in  con- 
tractions of  the  dystrophic  calf  muscles  tenotomy  of  the  Achilles  tendon  may 
be  of  use;  in  very  slowly  progressive  cases  with  suitable  topographic  distribu- 
tion of  the  still  healthy  and  of  the  diseased  muscles,  tendon  transplantation, 
in  "loose  shoulders"  fixation  of  the  scapula'  to  the  thorax  (scapulo-pexia)  or 
approximation  of  the  scapula;  to  one  another,  further,  the  correction  of 
scoliosis  by  supportive  apparatus,  etc.  From  a  prophylactic  point  of  view  I 
would  advise  you  to  take  to  heart  the  idea  that  every  intercurrent  disease  in 
dystrophic  patients,  even  the  slightest  one,  and  especially  bronchitides,  should 
be  treated  from  the  start  with  special  care. 

B.    The  Neural  Form 

This  variety  of  progressive  muscular  atrophy  is  characteristically  an 
heredo-family  disease.  "Erratic"  cases  are  quite  rare,  while,  on  the  other  hand, 
up  to  thirty  cases  have  been  counted  in  the  same  family.  The  male  members 
of  these  families  are  attacked  by  preference,  if  not  exclusively.  As  exciting 
causes,  infectious  diseases  and  overexertion  are  accused. 

This  form  is  also  called  "The  Charcot-Marie  type"  of  progressive  muscu- 
lar atrophy.  Besides  these  two  authors.  Tooth  and  Hoffmann  have  rendered 
service  in  establishing  the  clinical  picture  of  this  disease.  The  term  "neurotic" 
muscular  atrophy,  introduced  by  the  last,  should  naturally  be  corrected  into 
"neural,"  since  the  disease    has  not  the  least  thing  to  do  with  the  neuroses. 

Symptomatology,  Course  <ukI  Prognosis 

The  disease  begins,  as  a  rule,  between  the  sixth  year  of  life  and  puberty; 
however,  cases  have  lieen  known.to  begin  at  the  end  of  the  first  year  of  life,  or 
in   the  third  and  even  in  the  fourth  decade. 

The  disease  consists  in  a  slowly  progressive  muscular  wasting  which  begins 
Symmetrically  at  the  extremities  of  the  limbs,  to  attack  later  the  [eg  and  fore- 
arm.     Usually  the   feet   are  first  affected,  less   frequently   the  hands,  or  the  hands 

and    feet    simultaneously.     The   following  sympl s   are   produced:   atrophy 

of  the  smaller  muscles  of  the  feet,  the  peronei,  extensor  communis  digitorum, 

elub-foot    and    claw-toes,    atrophy    of    the    calf    muscles ;    wasting    of    the    small 
muscles  of  the  hand,   with  claw-hand,  later,  atrophy  of  the  muscles   of  the   fore- 


106  LECTURE    VI 

arm  with  eventually  loss  of  the  claw  position  of  the  fingers.  Usually  the  dis- 
ease halts  abruptly  at  the  level  of  the  knees  and  elbows ;  through  this  the  pic- 
ture of  "stork  legs"  can  occur.  In  advanced  stages  the  thigh  muscles  may  be 
also  attacked,  as  a  rule,  however,  only  the  vastus  interims.  One  can  always 
plainly  perceive  that  the  atrophy  is  the  primary  phenomenon,  the  paretic 
symptoms  only  its  consequence ;  these  are,  however,  not  rarely  very  slight,  also 
the  extremely  slow  progression  of  the  disease  admits  of  the  possibility  that  the 
still  retained  parts  of  the  muscles  may  adapt  themselves  functionally  to  the 
abnormal  condition.  One  of  my  patients  in  his  forty-fifth  year  could  still  cut 
tin. 

The  tendon  reflexes  decrease  and  finally  are  lost,  the  skin  reflexes  are 
nearly  always  normal.  Fibrillary  tremor,  whose  importance  in  the  picture  of 
spinal  progressive  muscular  atrophy  we  will  point  out  later,  is  also  frequent 
here,  but  in  no  way  constant.  The  mechanical  and  electric  irritability  of  the 
affected  muscles  decrease  and  are  finally  lost;  reaction  of  degeneration,  on 
the  other  hand,  is  almost  always  incomplete,  since  for  a  long  time  a  large  con- 
tingent of  normal  muscular  fibers  are  mixed  witli  the  degenerated  ones. 
Pseudo-hypertrophies  never  occur,  contractures  of  the  calf  muscles  are  very 
rare. 

In  contradistinction  to  the  other  forms  of  muscular  atrophy  it  must  be 
observed  that  sensibility  is  disturbed  in  many  cases,  botli  subjectively  and  ob- 
jectively.  One  or  more  of  the  following  symptoms  are  observed:  Paresthesias, 
lancinating  or  cramplike  pains,  sensitiveness  to  pressure  of  the  nerve  trunks, 
hypesthesia  of  the  integuments  (usually  slight)  of  the  peripheral  portions  of 
the  extremities,  reduction  or  loss  of  vibratory  sensation  in  the  same  territory, 
disturbances  of  sense  of  position,  ataxic  phenomena.  Vasomotor  disturbances 
also  occur,  as  hyperidrosis  of  the  feet,  hypothermia,  cyanosis,  and  marbling  of 
the  legs,  and  even  perforating  ulcer  of  the  foot !  If  cure  is  excluded,  never- 
theless, the  disease  cannot  only  halt  definitely  at  the  elbows  and  the  knees,  but 
may  be  interrupted  in  its  evolution  by  year  long  pauses.  Although  in  general 
there  is  great  loss  of  resistance  to  intercurrent  diseases,  especially  to  those  of 
the  lungs,  nevertheless,  many  of  these  patients  live  to  be  old  people. 

Pathological  A nntomy 

According  to  the  anatomical  substratum  of  "neural  progressive  atrophy" 
this  nosological  characterization  must  appear  entirely  insufficient  in  almost  all 
cases,  since,  as  a  fact,  along  with  the  neural  lesions  (degeneration  of  the 
peripheral  nerves,  usually  also  of  the  anterior  and  posterior  roots  as  well  as 
of  the  spinal  ganglia)  anil  the  degenerative  muscular  wasting  dependent  upon 
them,  there  are  in  almost  all  cases  which  come  to  autopsy  spinal  cord  changes 
in  addition,  as  I  said  at  the  beginning  of  this  lecture.  These  arc  degenerations 
in  the  posterior,  and  often  also  in  the  lateral  columns  of  the  cord.  Since  the 
degenerative  processes  reach  their  maximum  at  the  periphery  and  grow  less 
toward  the  center,  it  is  possible,  as  some  authors  affirm,  that  the  spinal  altera- 
tions are  only  secondary. 


THE    PROGRESSIVE    MUSCULAR    ATROPHIES  107 


Treatment 

The  treatment  to  be  recommended  in  neural  progressive  muscular 
atrophy  agrees  in  the  main  with  that  of  the  spinal  form  which  will  be  discussed 
at  the  end  of  this  lecture.  In  the  neural  type,  however,  on  account  of  its  re- 
lative mildness  and  its  exceedingly  slow  development  a  better  result  may  be 
promised  from  surgical  treatment  (arthrodesis,  tendon  transplantation,  tenot- 
omy) and  on  this  account  it  can  find  more  frequent  use. 

G.    The  Spinal  Form 

In  considering  this  third  chief  form  of  the  progressive  muscular  atrophies 
I  will  confine  myself  to  the  type  described  about  the  middle  of  the  last  century 
by  Aran  and  Duchenne,  not  considering  the  exceedingly  rare  cases  which  have 
been  denominated  the  Werdnig-Hoffmann  type.  This  last  is  distinguished  from 
the  Aran-Duchenne  type  by  beginning  in  early  childhood,  terminating  in  from 
one  to  six  years  through  broncho-pneumonia,  or  by  extension  of  the  process  to 
the  medulla,  attacking  the  muscles  of  the  pelvis,  the  trunk  and  the  thigh,  and 
corresponding  to  the  rapid  extension  of  the  atrophy  presenting  a  complete  re- 
action of  degeneration.  Further  the  Werdnig-Hoffmann  type  is  almost  always 
characterized  by  hereditary  family  occurrence,  while  the  classical  Aran- 
Duchenne  type  usually  appears  as  an  isolated  phenomenon.  On  this  ac- 
count it  takes  a  special  position  in  the  circle  of  the  progressive  muscular 
atrophies,  since  in  it  the  role  of  the  endogenic  factors  appears  to  be  less  pre- 
ponderant. Also  other  facts  give  the  impression  that  here  exogenic  factors  act 
to  a  somewhat  more  definite  degree. 

Etiology 

As  a  fact  it  is  striking  that  people  of  the  working  classes  are  to  a  so  pre- 
ponderant extent  victims  of  this  disease,  much  more  so,  indeed,  than  would  be 
accounted  for  by  the  numerical  excess  of  this  class  of  society.  The  thought 
that  tlu'  evil  influence  of  physical  excesses  is  much  rather  to  be  held  responsible, 
gains  probability  from  the  finding  that  the  atrophy  begins  usually  on  the  right, 
in  right-handed  people,  on  the  left,  in  left-handed  ones.  Smiths  and  weavers 
are  specially  predisposed.  As  atypical  localization  of  the  initial  symptoms  they 
have  been  known  to  begin  in  tin  calves  in  a  ballet-dancer,  in  the  right  deltoid  in 
a  ribbon-weaver  who  had  to  hohl  his  right  arm  up  continually,  in  the  muscles 
of  the  radial  side  of  the  forearm  in  a  grave-digger,  etc.  Very  important  is  the 
circumstance  that  an  acute  poliomyelitis  .anterior  in  childhood  seems  to  furnish 
a  locus  minoris  resistentiffi  which  can  predispose  to  the  later  outbreak  of  a 
spinal  progressive  muscular  atrophy.  Finally,  the  not  rare  history  of  previous 
traumatic  influences  and  acute  infectious  diseases  is  to  lie  mentioned,  while  the 
liases  impressing  themselves  clinically  as  progressive  muscular  atrophy,  but 
which  depend  upon  syphilis,  form  an  affection  by  themselves  which  can  usually 
h^  brought  to  a  standstill  by  antisyphilitic  treatment. 


108  LECTURE    VI 

Course  and  Symptomaiology 

The  beginning  of  the  disease,  which  usually  occurs  between  the  twenty-fifth 
and  forty-fifth  year  and  affects  especially  men,  is  gradual  and  little  marked.  A 
thinning  of  the  small  muscles  of  the  hand,  namely  those  of  the  ball  of  the  thumb 
which  is  already  plain,  usually  first  makes  the  patient  aware  of  his  disease.  The 
hand  attacked  first  (as  already  said,  usually  the  right)  even  later  remains 
ahead  of  the  other  in  the  intensity  of  the  muscular  wasting.  After  the  abductor 
pollicis  brevis,  opponens,  flexor  pollicis  brevis  and  abductor  pollicis  one  by  one 
are  affected  by  the  atrophy  (''individual  atrophy*')  the  first  metacarpal  bone 
is  pulled  backward  through  the  preponderant  action  of  the  extensor  pollicis 
longus,  so  that  the  thumb  comes  to  lie  on  the  same  plane  with  the  other  fingers. 
This  is  the  so-called  "Ape-hand,"  which  by  the  atroph}'  of  the  interossei  later 
becomes  a  "claw-hand"  and,  still  later,  when  the  deep  and  superficial  flexors 
and  the  hypothenar  muscles  have  become  atrophied,  a  "skeleton  hand."  It  is 
now  typical  of  the  further  course  of  the  affection  that  the  atrophy  proceeds 
farther  "by  leaps."  After  the  forearm  muscles  or  indeed,  before  them,  the  mus- 
cles of  the  shoulder-girdle  are  affected,  not  those  of  the  arms.  The  shoulders 
become  "loose,"  the  arms  dangle  like  flails  from  their  insertion.  Little  by 
little,  however,  the  arms,  as  well  as  the  trunk  muscles,  are  affected,  when  in 
order  to  maintain  the  equilibrium  the  physiological  curves  of  the  spinal  column 
become  exaggerated.  Finally,  the  entire  upper  half  of  the  body  may  look  like 
that  of  a  skeleton.  On  the  other  hand  the  lower  limbs  are  either  attacked  not 
at  all,  or  only  in  the  last  stages  of  the  disease  (with  very  rare  exceptions). 
The  clavicular  part  of  the  trapezius  as  "ultimum  moriens"  usually  remains 
longest  intact  of  the  shoulder  muscles,  while  the  muscles  of  the  neck  are  usually 
not  attacked  at  all. 

As  opposed  to  the  above  sketched,  comparatively  stereotyped  course,  com- 
mencement in  the  shoulder  muscles  marks  the  most  frequent  atypical  form. 

As  terminal  symptom-complexes,  which,  however,  only  develop  in  a  small 
number  of  cases,  I  would  mention  1,  affection  of  the  respiratory  muscles,  es- 
pecially of  the  diaphragm ;  2,  the  occurrence  of  bulbar  paralysis,  which  is  to 
be  described  in  the  next  lecture. 

The  clinical  characters  of  the  muscular  affection  are  in  the  Aran-Duchcnne 
disease  on  the  whole  quite  homogeneous.  Above  everything  it  is  to  be  em- 
phasized that  the  atrophy  is  the  primary  condition,  the  paralysis  only  its  result; 
the  earliest  functional  symptom  is  an  increased  fatigability.  With  wasting 
of  the  muscles,  sometimes  preceding  this,  the  phenomenon  of  "fibrillary  con- 
tractions" (see  page  64)  becomes  evident,  in  the  thenar  muscles  sometimes  so 
marked  that  a  motor  effect  (twitching  of  the  thumb)  occurs.  In  the  atrophic 
region  the  tendon  reflexes  are  diminished,  finally  lost.  Electric  irritability  is 
reduced,  but  the  reaction  of  degeneration  is  usually  only  incomplete  (slow  con- 
traction to  galvanism,  retained  faradic  irritability).  Also  it  is  rather  hard  to 
find  places  in  which  it  can  be  obtained,  since  there  is  almost  always  a  very  con- 
siderable number  of  normal  fibers  mixed  with  the  degenerated  ones,  even  to  the 
advanced  stage,  and  these  mask  the  anomalies  of  contraction.  The  reaction 
is  found  most  frequently  in  the  ball  of  the  thumb.     After  the  changes  in  the 


THE    PROGRESSIVE    MUSCULAR    ATROPHIES  109 

upper  extremity  first  affected,  which  have  preponderated  for  some  time,  are 
overtaken  by  those  in  the  other  arm,  the  disease  usually  progresses  symmetri- 
cally, and  indeed,  so  gradually,  that  by  practice  (the  development  of  new- 
synergies,  etc.),  the  patients  are  long  able  to  use  their  skeleton  arms  for  some 
acts  astonishingly  well. 

Apart  from  occasional  sensitiveness  to  pressure  in  the  wasting  muscles  and 
from  the  paresthesias  which  the  dead  weight  of  the  hanging  extremity  dragging 
upon  the  brachial  plexus  produces,  sensibility  remains  intact.  Vasomotor  dis- 
turbances in  the  form  of  coldness,  marbling,  lividitv  of  the  integuments  occur 
quite  frequently,  more  rarely  there  is  firm  oedema  of  the  hands.  There  is  often  a 
tendency  to  profuse  sweating.  Of  trophic  phenomena  loss  of  subcutaneous 
fat.  thinning  of  the  skin  over  the  atrophic  parts,  as  well  as  the  very  infrequent 
atropines  of  bone,  and  arthropathies  are  to  be  mentioned.  The  sphincters  and 
the  innervation  of  the  genitals  always  remain  normal. 

Pathological  Anatomy 

There  is  a  progressive  sclerotic  atrophy  of  the  cells  of  the  anterior  horns 
of  the  spinal  cord  with  pigment  deposit  in  them,  in  the  segments  corresponding 
to  the  affected  muscles;  with  this  there  is  of  course  atrophy  of  the  peripheral 
motor  neurone  as  well  as  the  picture  of  secondary  degeneration  of  muscles  (  re- 
duction of  the  size  of  the  fibrilli,  granular  or  fatty  degeneration  of  the  sarco- 
plasm,  empty  sarcolemma  sheaths  with  increase  of  nuclei,  proliferation  of  the 
connective  tissue  of  the  septa,  finally  moderate  fat  infiltration  and  a  few 
hypertrophic  fibers). 

Differential  Diagnosis 

Progressive  dystrophy  begins  at  a  much  earlier  age  than  spinal  progressive 
muscular  atrophy,  is  usually  a  family  disease,  leaves  the  hands  uninvolved,  is 
often  accompanied  by  pseudo-hypertrophies,  never  with  fibrillary  contractions, 
etc.;  neural  progressive  muscular  atrophy  begins  in  childhood  at  the  peripheral 
ends  of  the  upper  as  will  as  of  the  lower  extremities,  does  not  extend  to  the 
roots  of  the  extremities,  is  a  family  disease. 

The  professional  pareses  (see  above.  page  .'i ) .  drummer's  paralysis,  cigar- 
maker's  paralysis,  etc.,  can  lead  to  an  "individual  atrophy"  of  the  small  hand 
muscles,  which,  however,  the  cause  ceasing  to  act,  soon  present  not  a  pro- 
gressive, but  rather  a  regressive  character.  On  account  of  the  frequent  uni- 
lateral commencement  of  spinal  muscular  atrophy,  these  conditions  must  al- 
ways lie  kept  ill  mind  in  differential  diagnosis.  The  same  remarks  apply  alsi> 
In  certain  rare  forms  of  lead  paralysis,  in  which  the  small  hand  muscles  are 
albeted  with  paralysis  and  atrophy  before  the  radial  muscles.  The  antecedents 
of  the  patient  (occupation,  lead  colic)  and  the  objective  signs  of  saturnism 
(blue  hue  on  (he  gums,  tremor,  hard  pulse,  anemia)  are  to  be  looked  for.  Still 
other  intoxications  (arsenic,  alcohol,  bisulphide  of  carbon)  produce  similar 
clinical   pictures. 

The  differential  points  between  this  disease  and  amyotrophic  lateral  sclero 
sis,    syringomyelia    and    spinal    gliosis,    spondylitis    and    hypertrophic    cervical 


110  LECTURE    VI 

pachymeningitis  are  furnished  by  the  difference  in  the  reflexes  and  in  the  sen- 
sibility.    They  will  be  described  in  connection  with  these  diseases. 

At  this  point  we  might  also  consider  the  so-called  "chronic  anterior  polio- 
myelitis," a  disease  whose  separation  from  spinal  progressive  muscular  atrophy 
is  still  sub  judice.  It  is  a  rare  disease  of  mature  age,  which  is  possibly  of  auto- 
toxic  origin — for  example,  a  number  of  cases  in  diabetics  have  come  under  ob- 
servation. The  disease  begins  usually  in  the  legs,  more  rarely  in  the  muscles 
of  the  shoulder-girdle,  as  weakness  which,  little  by  little,  passes  over  into  paraly- 
sis and  progresses  by  no  means  so  slowly  as  the  Aran-Duchenne  disease.  Ex- 
tensive segments  of  the  extremities  are  attacked  simultaneously,  reaction  of 
degeneration  and  paresis  or  paralysis  precede  the  atrophy,  and  its  extension 
is  not  by  leaps.  The  disease  can  come  to  a  standstill,  indeed  recoveries  have 
been  observed.  On  the  other  hand  a  relatively  rapid  fatal  termination  (in  from 
1  to  3  years)  as  a  result  of  asphyxia,  aspiration  pneumonia,  etc.,  is  to  be  feared. 

Prognosis  and  Treatment 

The  prognosis  of  progressive  spinal  muscular  atropny  as  regards  recovery 
or  even  as  to  definite  standstill,  is  altogether  bad.  Nevertheless  the  progres- 
sion of  the  disease  is  almost  always  a  very  slow  one  extending  over  decades, 
and  a  direct  menace  to  life  through  bulbar  paralysis  or  respiratory  failure  is 
comparatively  rare.  The  treatment  can  only  be  inspired  by  the  hope  of  slow- 
ing and  stopping  the  process  for  a  time  and  of  supporting  functional  compen- 
sation. Moderate  and  careful  exercise  and  massage  of  the  muscles  not  yet 
affected  is  the  most  important  thing;  also  labile  electrical  treatment  with  weak 
currents,  the  cathode  on  the  affected  muscles,  can  act  favorably.  I  would  warn 
you,  however,  against  all  energetic  measures,  also  against  faradization,  and 
advise  you  above  everything  to  leave  the  already  affected  muscles  entirely  at 
rest.  Further,  galvanization  of  the  spinal  cord,  the  technique  of  which  will  be 
considered  in  the  next  lecture  will  come  into  consideration.  As  to  drugs, 
strychnine  injections  (0.0015  (gr.  1/40)  once  a  day)  may  be  tried  according 
to  Goiters'  recommendation  ;  the  formerly  much-vaunted  silver  nitrate  and  ergot 
cures  are  certainly  entirely  useless.  Bath  cures  and  hydrotherapy  also  ac- 
complish nothing;  when  the  patient  demands  them  one  should  limit  himself 
according  to  the  motto  "non  nocere,"  in  that  care  is  taken  to  avoid  excesses, 
and  above  everything,  cold  water  treatment. 


LECTURE    VII 

While  in  the  last  lecture  we  have  become  acquainted  with  clinical  pictures 
whose  anatomical  substratum  has  its  location  partly  in  the  peripheral  (spino- 
muscular)  motor  neurones,  partly  in  their  end  apparatus  the  muscles,  we  will 
turn  our  attention  to-day  to  degenerative  affections  in  which  the  central  (cor- 
tico-spinal)  motor  neurones  are  affected  by  the  disease  process.  Since  this 
occurs  with  avoidance  of  neighboring  but  physiologically  different  fiber  sys- 
tems, that  is  in  elective  manner,  we  speak  of  "system  diseases"  in  contrast  to 
the  "diffuse  diseases"  of  the  nerve  centers  (as,  for  example,  inflammatory  af- 
fections, multiple  sclerosis,  syringomyelia,  etc.). 

We  will  begin  with  the  study  of  the  so-called  spastic  spinal  paralysis,  al- 
though this  clinically  very  typical  disease  picture,  as  I  will  remark  in  advance, 
is  not  to  be  considered  as  the  expression  of  a  unique  and  definite  disease  of  the 
spinal  cord,  but  rather  presents  a  syndrome  which  occurs  in  the  course  of  a 
heterogeneous,  but  without  exception,  organic  set  of  spinal  affections,  either  to 
establish  itself  definitely  or  to  pass  over  into  other  symptom  complexes. 

Spastic  Spinal  Paralysis 

In  187-5  and  1876  Erb  and  Charcot,  independent  of  one  another,  under  the 
respective  designations  of  "Spastische  Spinalparalyse"  and  "Tabes  dorsal 
Spasmodique,"  described  for  the  first  time,  a  disease  condition  which  they  cor- 
rectly  suspected,  and  which  we  to-day  know  certainly,  to  depend  upon  more  or 
J'--  symmetrica]  lesions  of  tic  spinal  pyramidal  tracts,  in  whose  territory 
cortico-spina]  conduction  is  broken.  Although  indeed  it  was  originally  as- 
sumed thai  this  must  be  always  an  elective  degeneration,  a  system-disease,  of 
that  neurone,  later  experience  has  proved  the  untenableness  of  this  view;  so, 
for  example,  compression  of  the  spinal  cord  (by  a  tumor  an  exotosis  of  the 
vertebral  column,  etc.)  can  manifest  itself  for  a  long  time  only  by  symptoms 
of  involvement  of  the  pyramidal  tract,  in  other  words  by  the  phenomena  of 
spastic  spinal  paralysis.  The  same  thing  occurs  in  "multiple  sclerosis,"  which 
is  characterized  by  the  dissemination  of  anas  of  proliferated  glia  throughout 
all  the  nerve  centers,  also  in  many  brain  diseases,  for  example  in  internal  hydro- 
cephalus. It  hence  seems  as  if  the  pyramidal  tracts,  mainly  in  their  distal  por- 
tions, arc  easily  disturbed  in  their  integrity  by  the  most  varied  pathological 
conditions. 

On  this  account  one  must  always  lie  aware  that  in  making  what  is,  as  we 
will  sic,  the  exceedingly  easy  diagnosis  of  "spastic  spinal  paralysis"  he  is  men- 
tioning only  a  syndrome,  not  a  disease,  and  should  investigate  industriously 

111 


112  LECTURE    VII 

and  carefully  further,  if  new  symptoms  do  not  present  the  situation  in  another 
light.  The  clinical  picture  usually  unfolds  itself  so  slowly  and  gradually  that 
only  after  years  does  it  unmask  itself  as  an  expression  of  one  of  the  disease 
conditions  mentioned  above.  There  remain  finally,  however,  as  Minkowsky, 
Bernhardt,  Dejerme,  Schultze,  and  others  have  shown,  still  cases  which  prove 
to  be  due  to  a  purely  systemic  degeneration  of  the  cortical-spinal  tract  in 
its  spinal  portion,  a  degeneration  with  which  a  decided  reactive  or  com- 
pensatory glia  proliferation  (sclerosis)  goes  hand  in  hand.  Indeed,  we  have 
learned  through  St  rum  pell  that  true  spastic  spinal  paralysis  can  occur  as  a 
hereditary  family  disease,  a  proof  that  it  may  depend  upon  endogenic  factors. 
A  reduced  power  of  resistance  of  the  pyramidal  tracts  from  the  start,  perhaps 
also  plays  a  role  in  those  forms  which  are  set  up  by  exogenic  factors;  for  ex- 
ample, anemia,  carcinosis,  syphilis,  the  puerperium,  lead  poisoning,  lathyrism 
(chronic  intoxication  hy  the  use  of  spoiled  chick  peas — lathyrus  cicera  and 
L.  sativa).  All  these  factors,  however,  frequently  lead,  instead  of  to  isolated 
pyramidal  degeneration  to  simultaneous  involvement  of  the  lateral  and  posterior 
columns,  the  so-called  system  diseases  in  which  motor  and  sensory  phenomena 
are  united.  This  last  is  also  the  case  in  pellagra,  an  intoxication  from  spoiled 
corn-Hour  endemic  in  Lomhardy,  in  Roumania  [and  of  recent  years  observed 
in  the  United  States — Translator]. 

Symptomatology 

In  full  development  of  "spastic  spinal  paralysis"  there  are  in  both  legs,  only 
in  a  small  number  of  cases  also  in  the  arms,  the  anomalies  which  we  denominate 
the  spastic  symptom  complex,  and  which  pathologically  are  to  be  referred  to 
the  elimination  of  the  function  of  the  pyramidal  tracts  in  a  longer  or  shorter 
portion  of  their  spinal  course. 

Physiology  of  the  Pyramidal  Tracts 

The  pyramidal  tracts  or  cortico-spinal  tracts  have,  as  is  known,  their  origin 
in  the  motor  region  of  the  cerebral  cortex.  They  pass  through  the  internal 
capsule,  the  crus  cerebri  and  the  pons  to  the  medulla.  There  a  separation  takes 
place  into  the  lateral  pyramidal  tract  which  passes,  by  way  of  the  "decussa- 
tion of  the  pyramids,"  into  the  opposite  lateral  column  of  the  spinal  cord,  and 
the  anterior  pyramidal  tract  which  descends  on  the  same  side  in  the  anterior 
column.  Both  categories  of  fibers  end  about  the  cells  of  the  anterior  horn  at 
different  levels  of  the  spinal  cord,  those  of  the  anterior  pyramidal  tract,  how- 
ever, only  after  they  have  passed  through  the  anterior  commissure  of  the 
spinal  cord,  that  is,  have  crossed  to  the  opposite  side. 

Through  these  pyramidal  tracts,  now,  the  psycho-motor  impulse  is  trans- 
mitted from  the  cortical  motor  centers  of  the  cerebrum  to  the  spinal  centers 
of  the  different  muscles  of  the  body.  But  not  exclusively  by  this  path.  Rather 
are  these  chief  motor  tracts  assisted  in  their  task  by  a  number  of  accessory 
motor  tracts,  by  fiber  tracts  which  I  have  included  under  the  name  "sub-cortico- 
spinal  tracts";  their  points  of  origin  (the  red  nucleus,  the  optic  thalamus,  the 


SPASTIC    SPINAL    PARALYSIS  113 

roof  of  the  mid  brain,  Dcitcrs'  nucleus)   are  connected  with  the  motor  region 
f  the  cerebral  cortex,  so  that,  thanks  to  these  last,  even  on  elimination  of  the 
pyramidal  tracts,  part  of  the  psychomotor  impulses  can  be  transmitted  in  a 
roundabout  way  to  the  cells  of  the  anterior  horn  and  hence  to  the  muscles. 

Now,  however,  the  pyramidal  tracts  also  exercise  an  influence  upon  the  im- 
portant mechanism  of  the  regulation  of  the  tonus  already  mentioned  and  also 
upon  the  production  of  the  tendon  and  bone  reflexes.     They  exercise  an  in- 
hibition, in  that  they  take  care  that  reflex  action  and  the  degree  of  tonus  does 
QO      ,o  beyond  a  definite  and  useful  degree.     Without  this  inhibitory  action  of 
the  pyramids,  tonus  and  reflexes  would  not  present  such  constant  relations  as 
ually  the  case.     If,  however,  the  pyramids  are  destroyed,  hypertonia  and 
rreflexia  take  the  upper  hand  to  a  degree  hindering  motion,  so  that  the 
lis  cannot  be  sufficiently  relaxed  and  even  the  mere  jarring  of  the  body  in 
aotion  suffices  to  set  up  reflex  contractions. 

ifkr  this   short  physiological   introduction,  it   is   understandable   that   in 

I      quence  of  the  vicarious  action  of  the  subcortico  spinal  tracts*  the  affected 

extremities   arc  not  entirely  paralyzed,   but   only   more   or  less   paretic;   this 

>is,  however,  affects  nearly  all  the  muscles  of  the  limbs  in  question,  to  the 

extent.     In  the  paretic  muscles  a  spastic  rigidity  is  present,  as  we  can 

v   convince  ourselves  by  palpation,  by  testing  resistance  and  by  passive 

.  -merits.     On  the  other  hand  there  is  no  atrophy  of  the  affected  muscles — 

rt  from  an  atrophy  from  disuse  which  occurs  late  and  is  never  very  great — 

as  the  muscles  remain  in  spite  of  the  pyramidal  affection,  in  uninterrupted  con- 

■    <  tion  with  their  trophic  centers,  the  cells  of  the  anterior  horn.     For  the  same 

i      .mi,  along  with  anatomical  integrity  of  their  structure,  the  muscles  preserve 

their  electric  irritability. 

The  gait  of  such  patients,  in  consequence  of  the  paresis  and  rigidity  of  the 
.  i<  altered,  in  very  characteristic  fashion.     Since  the  rigidity  is  especially 
in  uked  in  the  glutei,  the  quadriceps,  the  adductors,  and  the  gastrocnemius,  the 
leg  is  extended  rigidly  at  the  knee  and  hip,  the  thigh  is  held  in  adduction,  the 
are  raised  from  the  floor  so  that  the  patient  sometimes  walks  only  on  the 
|       s  of  liis  feet,  and  in  order  to  prevent  falling  backward,  must  shift  his  center 
<>l  gravity  forward,  which  forces  him  to  make  use  of  a  walking-cane.     Locomo- 
-   carried  out  with  short,  audibly  dragging  and  careful  steps;  since  the 
Joints  are  held  by  the  muscular  rigidity  in  a  condition  of  extensive  "muscular 
ankylosis"  (Striimpell)  the  feet  can  scarcely  be  raised  from  the  floor,  but  must 
alternately  be  swung  forward  in   a  lateral   arc  one  around   the  other   ("heliko- 
podia"  "circumduction").     Sometimes  a  "sec-sawing"  of  the  body  at   every 
itep  is  noticeable.     In  long  duration  of  the  disease,  the  anomaly  of  position  of 
the  leg  is  more  and  more  fixed,  on  account  of  structural   shortening  of  the 
icrtonic  muscles  so  that  a  true  pes  equinovarus  can  develop.     Only  excep- 
tionally  are   there  contractures   in   flexion   at   the   knee   and   at    the  hi]),   an   oc- 
currence that  puts  an  end  to  the  patient's  ability  to  walk. 

In  the  rare  "ascending"  cases  of  spastic  spinal  paralysis,  in  which  after  the 

•Besides  this,  usually  only  the  lateral  pyramidal  tracts,  not  the  anterior  pyramidal  tracts, 

fectecl. 


114-  LECTURE    VII 

legs,  the  upper  extremities  are  also  affected,  the  arms,  on  account  of  t 
equal  distribution  of  rigidity  and  paresis,  usually  take  the  following  po 
adduction,  flexion  at  the  elbow,  wrist,  and  finger  joints,  pronation. 

The  "spastic  symptom  complex"  is  completed  by  anomalies  of  reflexes  and 
associated  movements.  The  exaggeration  of  the  tendon  and  bone  reflexes  in 
the  region  of  the  paretic  muscles  (which  is,  pathogenetically,  the  analogue  of 
hypertonia)  is  usually  very  great.  At  the  same  time  as  this  hyperreflexia  in 
the  affected  musculo-tendinous  apparatus,  there  can  be  demonstrated  (he 
phenomenon  known  as  "clonus"  ("trepidation  epileptoide"  of  the  French).  For 
example,  if  the  tendo  Achillis  is  suddenly  stretched  by  pushing  up  the  anterior 
portion  of  the  foot,  rhythmical  oscillations  of  the  foot,  which  last  as  lo 
the  tension  of  the  tendon  is  kept  up,  are  produced.  This  is  by  far  the  most 
frequent   clonus — "ankle   clonus."      "Patellar   clonus"   cannot   infrequently   be 


Fig.  39. 
The  Bablnski  Reflex  (instantaneous  photograph). 

produced  by  suddenly  drawing  down  the  patella  and  attempting  to  hold  it  in  tin's 
position.  On  the  other  hand  "wrist,"  "finger"  and  "elbow  clonus"  are  rarely 
demonstrable.  The  behavior  of  the  skin  reflexes  is  much  less  constant  than  that 
of  the  tendon  reflexes;  the  plantar  reflex  is  found  exaggerated  with  a  certain 
degree  of  regularity,  but  the  degree  of  reflex  reply  to  skin  irritation  is  very 
difficult  to  estimate  as  to  its  pathological  indications.  All  the  more  valuable 
to  us  are  certain  abnormal  skin  reflexes  which  occur  when  the  pyramidal  triers 
are  interrupted.  Of  the  first  importance  is  the  Babinski  phenomenon,  in  which 
irritation  of  the  sole  of  the  foot  causes  a  rather  slow,  tonic  hypercxteiiMon  of 
the  great  toe  (see  Fig.  39),  sometimes  with  accompanying  plantar  flexion  01 
fanlike  spreading  of  the  other  toes.  In  the  second  place  stands  the  Opp  nlwim 
reflex,  in  which  the  same  reaction  is  produced  by  firmly  stroking  the  skin  on 
the  inner  surface  of  the  leg.  A  pathological  tendon  reflex  is  the  J 
BecMerew  phenomenon  ("dorsal  foot  reflex")  ;  in  lesions  of  the  pyramidal 


SPASTIC    SPINAL    PARALYSIS  115 

percussion  on  the  lateral  part  of  the  dorsum  of  the  foot  (over  the  4th  and  5th 
metatarsal  bones)  may  cause  plantar. flexion  of  the  toe.s ;  normally  there  is  either 
no  reflex  or  extension  of  the  toes. 

That  the  "pathological  reflexes"  occur  normally  in  children  in  the  early 
months  of  life,  depends  most  probably  upon  the  fact  that  the  pyramidal  tracts 
develop  their  full  function  only  after  their  axis-cylinders  have  taken  on  their 
medullary  sheaths  in  the  course  of  the  early  part  of  the  post-embryonal  period. 
A  satisfactory  pathological  explanation  of  these  phenomena  is  wanting.  We 
can  at  most  conceive  that  the  reflex  irritation,  if  it  is  not  inhibited  from  above 
(that  is,  from  the  cerebrum)  breaks  into  paths  otherwise  closed  to  it.  The 
same  may  be  the  case  with  the  voluntary  impulse  intended  to  produce  a  definite 
movement  of  the  paretic  muscles  so  that  it  comes  to  set  up  unintended  "asso- 
ciated movements."  When,  for  example,  the  leg  cannot  be  drawn  up  on  the 
body,  without  decided  dorsal  flexion  of  the  foot  occurring  at  the  same  time,  we 
speak  of  the  tibial  phenomenon  or  "Striimpeirs  sign" ;  compulsory  hyperex- 
tension  of  the  big  toe  under  the  same  conditions  furnishes  the  toe  phenomenon; 
in  the  hand,  the  radial  phenomenon  occurs  (compulsory  dorsal  flexion  of  the 
hand  with  palmar  flexion  of  the  fingers),  further  the  pronation  phenomenon 
(compulsory  pronation  when  the  forearm  is  bent),  etc. 

Sensory,  vasomotor  and  trophic  disturbances,  also  anomalies  in  the  empty- 
ing of  the  bladder  and  rectum,  as  well  as  those  of  the  sexual  functions,  do  not 
belong  to  the  picture  of  spastic  spinal  paralysis. 

Course  (iiiil  Prognosis 

The  disease  begins,  as  a  rule,  gradually  and  little  marked,  at  first  with  only 
subjective,  often  but  temporary  difficulties;  after  long  walking  the  patient  feels 
weakness  and  sense  of  tension  in  the  legs,  symptoms  which  can  increase  to  a 
"spinal  intermittent  limping"  (Dcjcrhu)  which,  however,  for  a  long  time  dis- 
appear again  when  the  patient  is  rested.  Only  later  do  they  become  permanent 
and  then  the  objective  symptoms  of  the  disease  grow  more  and  more  plainly 
apparent.  If  the  clinical  picture  does  not,  as  is  usually  the  case,  unfold  itself 
as  the  initial  stage  of  some  other  disease  more  rich  in  symptoms,  but  definitely 
restricts  itself  to  the  spastic  symptom-complex,  the  progression  is  usually  a 
very  slow  one,  so  that  the  patients  may  remain  capable  of  movement  for  ten  or 
twenty  years  or  more,  indeed,  it  may  conic  to  a  stop  when  the  disease  lias  made 
relatively  hut   little  progress.      The  prognosis  as  to  life  is  hence  not   unfavorable 

in  the  true  spastic  spinal  paralyses,  which  usually  commence  between  the  ages 
of  20  and  40  years;  only  when  the  patients  (generally  on  account  of  con 
tractures  in  flexion  of  the  legs)  have  become  bedridden,  it  is  impaired  by  the 
danger  of  hypostatic  pneumonia.  As  to  the  prognosis  of  the  symptomatic 
form,  no  genera]  statement  can  be  made.  It  depends  naturally  upon  the  nature 
of  the  disease  causing  the  syndrome  "spastic  spinal  paralysis";  fur  example, 
where  it  depends  upon  compression  of  the  cord  by  a  tumor  which  .can  lie  re- 
moved by  operation,  cure  is  possible. 


116  LECTURE    VII 


Treatment 


In  the  very  first  place  it  is  necessary  to  warn  these  patients  against  ex- 
hausting gymnastic  exercises  in  which  many  of  them  are  inclined  to  seek  cure, 
and  to  make  clear  to  them  the  necessity  of  prolonged  rest  and  self-care.  The 
rigidity  can  very  often  be  alleviated  by  careful  massage  and  passive  movements ; 
protracted  tepid  baths  work  in  the  same  way.  The  practice  of  active  move- 
ments is  best  carried  out  in  the  bath,  since  the  influence  upon  their  weight  which 
the  limbs  experience  on  account  of  the  buoyant  effect  of  the  water  (this  can  be 
increased  by  the  addition  of  salt)  aids  greatly  in  the  work  to  be  accomplished, 
while  on  the  other  hand  the  diminution  of  the  stimuli  from  the  skin  acts  to 
some  extent  in  reducing  the  tonus.  On  account  of  the  long  duration  of  the 
disease  drugs  should  be  used  but  sparingly ;  though  occasional  courses  of 
bromides  or  scopolamin  (for  dosage  see  Parkinson  s  disease,  page  89)  can  be 
recommended  without  hesitation.  Faradization  is  strongly  contraindicated ;  if 
stabile  galvanization  of  the  rigid  muscles  or  their  nerves  or  the  "galvanization 
of  the  spinal  cord"  (two  large  electrodes,  one  on  the  neck  and  one  in  the  sacral 
region,  gradual  introduction  of  a  current  of  a  few  milleamperes,  let  it  pass  for 
5  minutes,  slowly  reduce  it,  change  the  poles  and  repeat  the  procedure)  is  of 
any  use  is  questionable ;  in  any  case  it  does  no  harm.  In  contractures,  ortho- 
pedic surgery  is  to  be  considered  (tenotomy,  supportive  apparatus).  The 
most  modern  surgical  procedure  recommended  for  spastic  paresis  is  "Rhizo- 
tomia  posterior"  proposed  by  Forster.  Based  upon  the  idea  that  the  produc- 
tion of  tonus  depends  upon  peripheral  stimuli  which  are  transmitted  through 
the  posterior  roots  of  the  spinal  cord  to  the  cells  of  the  anterior  horn  (see  page 
8)  several  pairs  of  these  roots  are  resected  in  order  to  make  movements  more 
easy  bv  getting  rid  of  the  rigidity.  We  will  consider  this  operation  again  later 
in  describing  Littlc^s  disease,  where  it  may  have  a  good  result  (provided  it 
is  followed  by  thorough  and  conscientious  gymnastic  after-treatment).  In 
spastic  spinal  paralysis,  on  the  other  hand,  it  seems  to  promise  less.  In  the 
only  case  with  which  I  am  acquainted  from  my  own  observation,  it  remained 
without  effect. 

As  to  the  causal  treatment  of  the  forms  dependent  upon  exogenic  factors, 
we  need  not  consider  it  further  at  this  point;  the  therapy  of  nervous  syphilis 
will  find  a  connected  description  in  a  later  lecture. 

Amyotrophic  Lateral  Sclerosis  and  Progressive  Bulbar  Paralysis 

These  names  have  become  attached  to  two  symptom-complexes  which  do 
not  represent  different  diseases  at  all,  but  only  describe  a  different  localization 
of  one  and  the  same  affection;  in  the  one  case,  in  the  spina],  in  the  other,  in  the 
bulbar  nerve  centers.  This  affection,  however,  presents  itself  anatomically  as 
a  primary,  progressive  degeneration  of  the  whole  motor  tract  due  to  endogenic 
factors  (though  occurring  as  a  hereditary  family  disease  only  in  a  continually 
decreasing  number  of  cases). 

Parenchymatous  degeneration  of  the  neurones  with  reactive  proliferation 
of  the  supportive  tissues  lies  at  the  base  of  both  diseases.      In  amyotrophic 


SPASTIC    SPINAL    PARALYSIS 


117 


lateral  sclerosis  these  changes  affect  the  cells  of  the  anterior  horn  of  the  spinal 
cord  and  their  processes,  in  progressive  bulbar  paralysis  the  motor  nuclei  of 
the  medulla  (the  homologues  of  the  anterior  horn  cells).  Besides  this,  the 
fibers  of  the  pyramidal  tracts  going  to  the  diseased  spinal  and  bulbar  nuclei 
(cortico-spinal  and  cortico-bulbar)  degenerate  throughout  their  whole  extent. 

Indeed,  the  motor  intersegmental  fibers  of  the  antero-lateral  tract  in  the 
spinal  cord  and  the  cortical  fibra?  propria?  in  the  cerebrum  have  been  found 
altered. 

Both  disease  forms  are  rather  rare,  and  in  the  great  majority  of  cases  occur 
between  the  30th  and  the  55th  years  of  life,  only  very  exceptionally  in  youth 
or  in  childhood;  amyotrophic  lateral  sclerosis  on  an  average,  earlier  than  bulbar 
paralysis,  the  first  beginning  mainly  in  the  4th,  the  last  in  the  5th  or  6th  decades. 
As  exciting  causes  exposure  to 
cold,  exhausting  diseases  and  over- 
exertion, have  been  noted  here  and 
there.  It  is  often  found  in  amyo- 
trophic lateral  sclerosis  that  these 
have  affected  particularly  the  hand 
in  which  the  symptoms  of  the  dis- 
ease begins.  Here  and  there  pro- 
gressive bulbar  paralysis  also  has 
occurred  in  connection  with  severe 
fatiguing  of  the  muscles  of  the 
lips,  cheeks,  and  tongue  (in 
players  of  wind  instruments  and 
glass  blowers).  Any  special  pref- 
erence of  one  sex  over  the  other 
is  not  apparent.  The  unity  of 
the  two  disease  forms  is  indicated 
by  the  fact  that  every  amyo- 
trophic lateral  sclerosis  sooner  or 
later  is  combined  with  progressive  bulbar  paralysis,  provided  that  the  patient  is 
not  earlier  removed  by  some  intercurrent  disease.  That  this  statement  is  not  re- 
versed, that  is.  that  progressive  bulbar  paralysis  may  occur  without  spinal 
symptoms,  is  explainable  from  the  fact  that  it  puts  an  end  to  life,  and  hence 
to  the  further  extent  of  the  degenerative  process,  relatively  early. 


Alteration  of  the  Spinal  Cord  in  Amyotrophic 
Lateral  Sclerosis.     Weigert-Pal  Stain. 


'  Symptomatology 

In  sketching  the  symptomatology  we  will  take  as  an  example  a  typical  case 
which,  beginning  with  spinal  symptoms,  ends  with  bulbar  involvement,  and  which 
has  been  denominated  as  Charcot's  disease,  alter  the  great  neurologist  who  de- 
scribed it  for  the  first   time  in  1865. 

As  to  the  name  amyotrophic  lateral  sclerosis,  proposed  by  Charcot  himself, 
it  characterizes  in  a  happy  manner  the  clinical  picture  which  represents  the 
combination  of  two  syndromes  already  studied  by  us.  namely,  1,  that   of  spinal 


118  LECTURE    VII 

progressive  muscular  atrophy  (the  expression  of  degeneration  of  the  peripheral 
motor  neurone),  and  2,  that  of  spastic  spinal  paralysis  (that  is,  pyramidal 
tract — or  lateral  sclerosis,  an  expression  of  the  degeneration  of  the  central 
motor  neurone). 

The  disease  begins  in  the  upper  extremities  and  almost  never  in  both  at  the 
same  time.  The  right  hand  usually  shows  the  first  anomaly  and  in  the  progress 
of  the  disease  the  symptoms  generally  predominate  for  a  considerable  time  on 
the  one  side.  The  patient  first  notices  a  rapid  tiring  and  a  feeling  of  weakness, 
awkwardness  and  stiffness,  often  also  of  tension,  particularly  in  the  finer  move- 
ments ;  soon  objective  anomalies  appear,  before  anything  else  a  progressive 
wasting  of  the  small  muscles  of  the  hand.  The  abductor  pollicis  brevis  usually 
atrophies  first,  then  little  by  little  the  other  muscles  of  the  ball  of  the  thumb, 
the  hypothenar  muscles  and  the  interossei.  The  different  deformities  which  we 
met  in  the  last  lecture  in  spinal  muscular  atrophy  occur  here  in  an  analogous 
manner,"  namely  the  "ape-hand,"  "claw-hand,"  and  "skeleton-hand."  On  the 
other  hand,  the  paretic  symptoms  are  much  more  marked  than  in  the  Aran- 
Duchenne  disease,  and  the  paresis  is  accompanied  with  such  manifest  hyper- 
tonia that  in  the  early  stages  of  the  disease  there  may  be  active  contractures 
which  fix  the  finger  and  wrist  joints  in  flexion.  Also  spastic  adduction  of  the 
arm,  flexion  and  pronation  of  the  forearm  occur.  However,  the  spasticity  in  the 
upper  extremities  diminishes  again  with  the  progressive  atrophy  (this  soon 
affects  also  the  extensor  side  of  the  forearm  and  shoulders). 

As  long  as  the  muscles  needed  for  their  production  are  present,  exaggera- 
tion of  the  tendon  and  periosteal  reflexes  are  to  be  found ;  now  and  then  wrist 
clonus  also  can  be  elicited.  In  the  atrophic  muscles  fibrillary  contractions  are 
very  manifest.  As  in  progressive  spinal  muscular  atrophy  and  on  account  of 
the  same  conditions  (namely,  the  gradual  progress  of  the  degeneration  from 
fiber  to  fiber  in  the  individual  muscles)  reaction  of  degeneration  is  found  only 
in  places  (particularly  in  the  thenar  muscles)  and  only  in  incomplete  develop- 
ment ;  in  general,  faradic  and  galvanic  irritability  are  reduced  in  proportion 
to  the  degree  of  muscular  wasting. 

For  a  comparatively  long  time,  the  lower  limbs  remain  normal  except  for 
a  marked  increase  of  reflexes.  Little  by  little,  however,  the  patient  experiences 
a  weakness  and  tension  in  the  legs;  also  the  whole  spastic  symptom-complex, 
as  we  have  already  described  it  (see  page  112),  develops.  (Only  the  Babinshi 
and  Oppenheim  reflexes  are  comparatively  frequently  absent.)  Here,  in  con- 
tradistinction to  the  arms,  the  amyotrophic  component  is  entirely  subordinate 
to  the  spastic  one  and  sometimes  atrophy  of  the  muscles  of  the  leg  and  foot  oc- 
curs only  late  in  the  disease. 

Trophic,  sensory,  and  sphincter  disturbances  are  absent  during  the  whole 
of  the  disease.  On  the  other  hand,  the  last  stage  of  the  inexorably  progressive 
affection  is  a  period  of  grave  increase  in  the  symptoms  on  account  of  the  ad- 
dition to  them  of  progressive  bulbar  paralysis  (glosso-labio-pharvngeal  paraly- 
sis).    The  first  description  of  this  last  we  owe  to  Durhcnne  (1860). 

Its  prodromal  symptoms  are:  Slight  difficulty  in  speaking  and  in  the  other 
movements  of  the  tongue,  occasional  difficulty  in  swallowing,  inability  to  whistle, 
etc.     Little  by  little  the  disturbances  of  function  in  the  muscles  of  the  tongue 


SPASTIC    SPINAL    PARALYSIS  119 

and  lips,  of  mastication,  of  swallowing  and  of  the  respiratory  muscles  become 
more  marked.  The  speech  becomes  continually  more  inarticulate.  The  pro- 
nunciation of  the  Unguals,  particularly  of  r  and  1,  is  first  affected;  soon  after 
that  of  the  labials  (  f,  w,  p,  b).  Among  the  vowels,  o  and  u,  whose  production 
is  favored  by  the  contraction  of  the  orbicularis  oris,  are  worst  articulated.  On 
account  of  involvement  of  the  muscles  of  the  velum  palati  there  is  difficulty  in 
the  formation  of  the  gutturals  (g  and  k)  ;  this  also  gives  the  voice  a  nasal  sound, 
since  the  nasal  cavities  can  no  longer  be  shut  off  from  the  pharynx  and  act  as 
resonance  chambers.  Besides  this,  the  speech  becomes  monotonous  on  account 
of  weakness  of  the  larynx  muscles.  The  paralysis  of  the  velum  palati,  as  well 
as  that  of  the  other  pharynx  muscles,  causes  appreciable  disturbances  in  swal- 
lowing; food,  particularly  that  of  a  liquid  character,  easily  gets  into  the  larynx 
and  produces  attacks  of  choking,  or  it  regurgitates  through  the  nose.  Chewing, 
on  account  of  the  progressive  paralysis  of  the  masseter  and  temporal  muscles, 
becomes  more  and  more  difficult ;  the  involvement  of  the  internal  pterygoid  makes 
itself  evident,  in  inability  to  draw  the  jaw  laterally  in  order  to  grind  the  food. 
The  taking  of  nourishment  is  rendered  difficult  by  still  other  factors;  the  tongue 
can  no  longer  shove  the  bolus  between  the  teeth  and  throw  it  back  again,  on 
which  account  it  remains  caught  between  the  gums  and  the  cheek ;  also,  it  can- 
not form  a  trough  to  direct  the  fluids  into  the  gullet,  hence  the  patient  when 
drinking  must  throw  the  head  backward.  The  saliva  also  is  imperfectly  swal- 
lowed and  flows  out  of  the  corners  of  the  mouth. 

Objective  examination  shows  that  the  functionally  so  seriously  affected 
muscles  are  also  attacked  by  degenerative  atrophy,  although,  corresponding 
to  the  condition  of  the  small  muscles  of  the  hand,  reaction  of  degeneration  is 
to  In  found,  as  a  rule,  only  in  a  few  muscles  (lips  and  tongue)  and  scarcely  ever 
in  complete  development.  The  tongue  is  flattened,  markedly  wrinkled,  as  if  its 
coating  of  mucous  membrane  had  become  too  large  for  it,  and  is  the  seat  of 
parked  fibrillary  contractions.  These  last  are  usually  very  evident  in  the  lips 
also,  while  here  the  atrophy  is  usually  concealed  on  account  of  fatty  deposit. 
Tin  velum  palati  hangs  down  like  a  thin,  sagging  curtain.  The  temporal  fossa 
presents  a  depression,  and  occasionally  fibrillary  contractions  are  to  be  ob- 
served in  the  masseters. 

The  masseter  reflex  is  usually  exaggerated.  The  palate  and  pharyngeal  re- 
flexes .-in'  almost  always  diminished  or  lost.  The  face  of  the  victim  of  bulbar 
mralysis  presents  a  characteristic  change.  The  lower  part  in  contradistinc- 
tion to  the  expressive  eyes  and  perfectly  movable  forehead,  is  of  mask-like  im- 
mobility :  the  mouth,  on  account  of  the  paresis  of  the  orbicularis  and  the  con- 
tracture  of  its  antagonists,  is  half  open,  and  extended  laterally  ("rire 
transversal")  or  its  angles  hang  down  deeply,  which  gives  to  the  physiognomy  a 
laciiiiiosc  expression.  The  skin  over  the  emaciated  muscles  of  the  checks  and 
chin  shows  exaggerated  folding,  only  very  exceptionally  is  the  upper  facial 
region  affected. 

This  condition,  which  the  mental  clearness  renders  specially  shocking, 
finally  terminates  either  by  vagus  paralysis  (permanent  severe  tachycardia, 
"Mi  weakness)  or  paralysis  of  the  diaphragm.  Every  bronchitis,  besides  this, 
contains  in   itself  the  greatest  danger  to  life,  since  coughing  anil  expectoration 


120  LECTURE    VII 

are  impossible.     Also,  the  danger  of  aspiration  of  particles  of  food  into  the 
lungs  cannot  indefinitely  be  avoided  in  any  of  these  patients. 

Course  and  Prognosis 

By  far  most  frequently  we  see  Charcot's  disease  begin  in  the  small  muscles 
of  the  hand  and  develop  further  in  the  manner  sketched  above;  only  very  ex- 
ceptionally are  the  muscles  of  the  shoulders  attacked  before  those  of  the  hand 
(scapulo-humeral  form).  Another  rare  method  of  beginning  is  that  the  spastic 
paralysis  of  the  lower  limbs  precedes  the  spastic  atrophic  manifestations  in  the 
upper  ones.  This  is  also  a  further  proof  that  spastic  spinal  paralysis  in  its 
later  course  presents  itself  as  only  a  partial  symptom  of  another  disease  process. 

Finally,  we  have  as  a  third  variety,  the  cases  which  begin  at  once  with  bulbar 
symptoms.  In  only  a  very  small  number  of  these  cases  an  extension  of  the 
motor  disturbances  to  the  muscles  of  the  neck,  the  shoulders  and  the  upper 
extremities  occurs ;  most  of  them  die  too  soon. 

The  prognosis  both  as  to  life  and  as  to  recovery  is  absolutely  unfavorable. 
Death  occurs  in  from  six  months  to  ten  years.  Relatively  the  best  prognosis 
is  given  by  the  form  beginning  as  paraplegia ;  by  far  the  most  unfavorable,  by 
primary  progressive  bulbar  paralysis,  of  which  no  case  lives  beyond  the  fifth 
year  of  the  disease,  only  few  beyond  the  second  year. 

Differential  Diagnosis 

In  amyotrophic  lateral  sclerosis,  the  resemblance  at  the  start  to  progres- 
sive spinal  muscular  atrophy  is  great ;  the  hyperreflexia  as  well  as  the  tendency 
to  contracture,  however,  enable  us  to  exclude  this  as  well  as  the  other  varieties 
of  progressive  muscular  atrophy;  also  the  progress  of  the  atrophy  is  much 
more  rapid  in  Charcot's  disease  than  in  that  of  Aran-Duchennc.  We  have  al- 
ready alluded  to  the  liability  to  confusion  of  the  paraplegic  form  of  amyo- 
trophic lateral  sclerosis  with  spastic  spinal  paralysis,  an  error  from  which 
reservation  of  opinion  until  the  disease  has  developed  further,  will  protect  us. 
Finally,  it  must  be  mentioned  that  an  affection  of  the  spinal  cord  which  we  will 
consider  in  the  next  lecture,  namely,  syringomyelia,  may  begin  with  atrophy  of 
the  small  muscles  of  the  hand  and  spastic  phenomena ;  however,  there  are  al- 
ways disturbances  of  sensibility  peculiar  to  it,  which  are  without  exception  ab- 
sent in  Charcot's  disease. 

As  to  the  glosso-labio-pharyngeal  paralysis,  an  acquaintance  with  the  clini- 
cal peculiarities  of  myasthenia  (see  page  9-1)  will  permit  distinguishing  the 
two  diseases  without  difficulty.  Similar  pictures  are  given  also  by  supranuclear 
pseudo-bulbar  paralysis  (see  Lecture  XV)  which  occurs  through  symmetrical 
interruption  of  the  neurones  passing  from  the  cortex  to  the  motor  nuclei  of  the 
bulb,  from  foci  of  disease  of  arteriosclerotic  origin.  These  occur,  however,  in 
connection  with  repeated  minor  apoplectiform  attacks  and  there  is  neither  mus- 
cular atrophy,  reaction  of  degeneration,  nor  fibrillary  contractions,  but  usually 
disturbances  of  intelligence  as  well  as  those  in  the  function  of  the  bladder. 

Further,   disease   pictures   resembling  those   of  bulbar  paralysis   occur  in. 


SPASTIC    SPINAL    PARALYSIS  121 

neuritis  of  the  motor  nerves  arising  from  the  medulla  (for  example,  after  diph- 
theria), also  from  acute  inflammatory  febrile  processes  (polioencephalitis  in- 
ferior), from  hemorrhages  and  softening  of  the  medulla  (embolism  and  throm- 
bosis of  the  basilar  artery)  beginning  acutely,  and  finally  from  compression 
of  the  medulla  (through  tumors,  caries  of  bone,  etc.).  In  these  last,  however, 
there  is  never  complete  symme'try  on  the  two  sides;  besides,  there  is  local  sensi- 
tiveness to  pain,  often  headaches  and  vomiting.  Also  further  symptoms  may 
be  found,  as,  for  example,  involvement  of  the  sensory  portions  of  the  trigeminus, 
of  the  acusticus,  etc. 

We  must  consider  here  "superior  bulbar  paralysis"  or  the  ophthalmoplegia 
chronica  progressiva  of  Graefe,  which  presents  an  analogue  of  progressive 
glosso-labio-pharyngeal  paralysis  and  may  be  combined  with  this.  This  is  char- 
acterized by  a  constantly  progressive,  gradual  atrophic  paralysis  of  the  differ- 
ent eye  muscles  which,  so  long  as  it  is  not  combined  with  glosso-labio-pharyngeal 
symptoms,  naturally  does  not  threaten  life.  Much  more  frequently,  indeed, 
progressive  ophthalmoplegia  is  not  a  definite  primary  degenerative  disease  like 
progressive  bulbar  paralysis,  but  a  part  of  different  organic  diseases  of  the 
brain  and  spinal  cord,  as,  for  example,  lues  cerebrospinalis,  tabes  dorsalis, 
dementia  paralytica,  sclerosis  multiplex,  etc.  There  are  also  neuritic  ophthal- 
moplegias ;  further,  acute  or  subacute  attacks  arising  upon  an  inflammatory 
basis  (polioencephalitis  superior)  as  well  as  those  which  are  caused  by  local 
processes  in  the  brain  and  at  the  base  of  the  skull  (for  example,  meningitides, 
tumors,  etc.). 

Treatment 

The  treatment  of  amyotrophic  lateral  sclerosis  comprises  that  of  its  com- 
ponents, spina]  progressive  muscular  atrophy  and  spastic  spinal  paralysis.  In 
progressive  bulbar  paralysis,  arsenic,  iodide  of  potassium  and  strychnine  are 
given,  although  a  favorable,  or  even  a  palliative  effect  of  these  medicaments  has 
not  been  recognized  with  certainty.  Of  electro-therapeutic  procedures  galvan- 
ization transversely  through  the  medulla  oblongata  (electrodes  the  size  of  a 
dollar  upon  both  mastoid  processes,  two  milleamperes  current  strength,  slowly 
introduced  and  reduced  again),  as  well  as  the  production  of  swallowing  move- 
ments, are  recommended.  (Anode  on  the  back  of  the  neck,  cathode  moved  up 
and  down  on  the  side  of  the  neck.)  We  can  counteract  the  flow  of  saliva  by 
atropin,  scopolamin,  or  duboisin  in  doses  of  1  J_.  nig.  (gr.  K2o)-  *s  long  as 
Bwallowing  is  appreciably  impaired  the  nourishment  should  be  half  liquid  and 
should  contain  as  many  calories  in  as  small  a  volume  as  possible  (nutritive 
preparations,  meat- juice,  etc.).  In  advanced  stages  tube  feeding  must  be  re- 
sorted to. 

In  order  to  aid  you  in  obtaining  a  comprehensive  view  of  the  different  forms 
of  primary  degeneration  of  the  motor  tracts  as  well  as  of  the  muscles  as  we  have 
learned  them  in  the  last  two  lectures,  I  have  prepared   tor  you  a  synoptic  table 

showing  these  affections  and  their  most  important  differentia]  characteristics. 


122 


LECTURE    VII 


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LECTURE   VIII 
The  Hereditary  Family  Ataxias 

Gentlemen:  Since  we  have  in  the  preceding  lectures  become  acquainted 
with  the  dyskinesias,  the  progressive  muscular  atrophies,  spastic  spinal  paraly- 
sis, amyotrophic  lateral  sclerosis  and  progressive  bulbar  paralysis,  a  series  of 
disease  conditions  which  characterize  themselves  by  preponderant  hereditary- 
family  occurrence,  we  will  consider  to-day  some  specially  interesting  representa- 
tives of  the  heredo-degenerative  nervous  diseases.  These  are  the  hereditary 
family  ataxias  which  since  their  clinical  groundwork  was  laid  by  the  great 
Heidelberg  clinician  Friedreich  (1861-1863),  have  always  held  medical  interest ; 
of  late,  however,  have  gained  in  actuality  in  that  alongside  of  the  typical  clini- 
cally and  anatomically  well-founded  picture  of  "Friedreich's  disease"  certain 
other  varieties  have  become  known.  We  will  first  make  the  acquaintance  of  the 
classical  type  of  the  disease. 

I.     Friedreich's  Disease  (Spinal  Heredo-ataxia) 

Sy  m  ptomat  ologi/ 

The  fundamental  symptom  of  this  disease,  which  begins,  as  a  rule,  in  child- 
hood, is  static  and  locomotor  incoordination  which,  from  a  scarcely  noticeable 
Beginning,  progressively  increases  to  the  highest  degrees  of  intensity,  and  al- 
most without  exception  affects  first  the  legs,  only  later  the  arms.  The  parents 
of  such  a  child,  which  usually  has  learned  to  walk  early  and  well,  but  as  time 
progresses  begins  to  complain  of  weariness  in  the  lower  extremities,  notice  that 
little  by  little  its  gait  becomes  uncertain,  spraddling  and  jerky.  Soon  a 
"wabbling  in  the  loins"  is  added  to  this  and  the  gait  comes  more  and  more  to 
resemble  that  of  a  drunken  man.  Finally,  the  excessive  degree  of  this  ataxia  no 
longer  permits  locomotion  and  the  patient  becomes  permanently  bedridden.  At 
the  same  time  a^  the  locomotor,  static  incoordination  has  also  developed:  even 
when  sitting  quietly,  the  body  sways  from  side  to  side,  each  limb  when  held 
free,  and  also  the  head,  oscillates  irregularly. 

This  ataxia  is,  as  we  may  assume  from  the  description  of  the  pathological 
anatomy  of  the  disease,  the  clinical  corollary  of  defeneration  in  the  posterior 
columns  and  in  the  spino-cerebellar  tracts  of  the  spinal  cord.  liotli  these 
systems  conduct  impulses,  which  belong  in  the  category  of  deep  sensibility  (see 
page  7),  and  which  have  come  by  way  of  the  peripheral  nerves  through  the 
posterior  roots  into  the  spinal  cord,  toward  the  brain.  While  now  one  portion 
of  these  impulses  passing  up  into  the  posterior  columns  and  proceeding  by  way 

1 2:5 


124  LECTURE    VIII 

of  the  optic  thalamus  to  the  cerebrum  exerts  a  quite  general  movement  regulat- 
ing action,  the  second  portion  which  passes  by  way  of  the  direct  cerebellar  tract 
and  column  of  Gowers,  to  the  cerebellum,  enters  into  the  special  function  of 
maintaining  the  equilibrium  on  standing  and  walking.  The  incoordination  in 
Frc'ul retch's  disease  is  now  a  mixed  form  of  these  two  types  of  ataxia.  The 
one  component,  which  is  to  be  considered  as  cerebellar  in  nature,  manifests  it- 
self by  severe  disturbances  of  equilibrium  which  we  have  just  mentioned,  through 
staggering  and  wabbling  on  locomotion,  through  swaying  of  the  body  also  while 
sitting  quietly.  We  will  have  an  opportunity  to  further  consider  these  symp- 
toms when  discussing  diseases  of  the  cerebellum. 

Exceedingly  frequent  further,  are  choreiform  movements,  which,  however, 
are  of  much  less  intensity  than  those  in  true  chorea.  Now  it  is  a  constant  play- 
ing of  the  fingers,  again  an  uniest  of  the  muscles  of  the  neck  and  face.  I  have 
occasionally  noted  it  only  in  the  upper  lid.  Athetoid  movements  have  been 
described  as  a  rare  occurrence. 

In  isolated  cases,  muscular  paralyses  have  developed  in  the  extremities  of 
patients  with  Friedreich's  disease.  It  appears  to  me  quite  doubtful  that  these 
are  uncomplicated  cases  of  the  disease,  since  in  one  such  case  of  my  own  I  was 
able  to  recognize  anatomically  the  combination  with  progressive  muscular 
atrophy,  while,  on  the  other  hand,  neuritic  processes  have  been  found.  Again, 
speecli  disturbances  are  among  the  most  regular  phenomena  in  spinal  heredo- 
ataxia ;  in  advanced  cases  they  are  almost  never  absent.  The  words  are  brought 
out  slowly,  with  difficulty,  and  often  in  a  slightly  scanning  and  explosive  manner 
(hesitating  speech).  A  frequent  changing  into  falsetto  (bitonality)  has  been 
pointed  out  by  Dejerine,  Thomas  and  myself.  Soca  has  noted  disturbances  of 
articulation  in  the  pronunciation  of  certain  letters,  namely  1,  k,  v  and  i. 

Among  disturbances  of  the  eye  muscles,  nystagmus  takes  the  first  place  on 
account  of  its  great  frequency.  In  the  early  stages  of  the  disease,  however,  it 
is  generally  absent.  Usually  it  appears  after  from  3  to  5  years'  duration  of 
the  affection,  sometimes  only  later;  in  but  very  few  cases  is  it  absent  entirely. 
As  a  rule,  it  is  a  dynamic  horizontal  nystagmus,  that  is,  made  up  of  rhythmical 
contractions  of  both  eyes  which  occur  in  the  horizontal  plane  and  are  set  up 
by  looking  to  one  side.  Almost  never,  on  the  other  hand,  do  we  find  a  static 
nystagmus,  that  is,  one  present  during  rest.  Paralyses  of  the  eye  muscles  are 
exceedingly  rare  in  Friedreich's  disease. 

The  tendon  and  bone  reflexes  are  diminished  in  the  early  stages,  entirely 
lost  later.  The  reflexes  in  the  lower  extremities  disappear  first,  in  the  upper 
extremities  only  some  years  later.  In  almost  all  cases  Babinski's  foot  phe- 
nomenon is  present  in  typical  form,  occasionally  a  slow  hyperextension  of  the 
big  toe  can  be  produced  also  by  stroking  the  inner  side  of  the  leg  (Oppcnheim's 
reflex).  In  general  the  skin  reflexes  are  undisturbed,  as  well  as  those  of  the 
mucous  membranes  and  of  the  pupil. 

Particularly  interesting  are  certain  deformities  which  are  typical  accom- 
paniments of  Friedreich's  disease.  A  peculiar  change  in  the  form  of  the  foot 
in  the  great  majority  of  cases  early  develops ;  the  dorsum  curves  upward  so  that 
the  foot  assumes  a  short,  compressed  and  concave  form  (pes  cavus)  ;  the  toes, 
particularly  the  great  toe,  become  permanently  held  in  dorsi-flexion  at  their 


THE    HEREDITARY    FAMILY    ATAXIAS 


125 


metatarsophalangeal  joints,  the  extensor  tendons  of  the  great  toe  give  the  im- 
pression of  being  considerably  shortened  and  stand  out  on  the  back  of  the  foot; 
finally?  the  foot  assumes  a  more  or  less  pronounced  equinus  position  (see  Fig. 
41).  Along  with  this  typical  deformity,  there  are  other  less  marked  varieties 
of  the  ''Friedreich's  foot."  In  the  production  of  this  deformity  the  '"balancing" 
action,  the  continual  contraction  on  the  one  hand  of  the  tibialis  anticus  and 
posticus  muscles,  and  on  the  other  of  the  muscles  of  the  calf  and  sole,  due  to 
the  ataxia  in  gait,  plays  a  great  part ;  besides  this — as  the  dorsal  con- 
traction of  the  great  toe,  which  may  be  considered  as  a  permanent  Babinski  re- 
flex, indicates — the  continuous  irritation  of  the  sole  on  standing  and  walking 
contributes  to  this  too.  I  have  seen  this  hyperextension  disappear  entirely 
again  after  the  patient  had  become 
bedridden.  Besides  this,  a  more  or 
less  marked  curvature  of  the  verte- 
bral column  occurs  in  time  in  most 
cases.  This  is  mainly  a  scoliosis, 
though  sometimes  a  kyphosis,  more 
rarely  a  lordosis.  A  satisfactory  ex- 
planation of  this  symptom  has  not 
3ret  been  given.  The  same  remarks 
apply  to  a  deformity  of  the  hand 
described  by  Friedreich  as  a  rarity, 
which  was  indicated  in  one  of  our 
cases  (permanent  hyperextension  of 
the   thumb). 

I  will  mention  also  some  unusual 
symptoms  of  Friedreich's  disease.  These  are  mainly  certain  sensory  disturb- 
ances. Diminution  of  the  sensibility  of  the  skin  occurs  only  in  the  latest 
stages,  for  example,  in  one  case  I  could  trace  its  first  beginning  only  after 
the  disease  had  existed  for  37  years.  It  occurs  chiefly  on  the  feet.  The 
muscular  sense  and  the  vibration  sense  can  be  affected  early,  but  in  most 
cases  remain  long  intact.  In  the  patient  mentioned  above  a  reduction  of 
stereo-esthesia  in  the  right  hand  occurred  first,  with  superficial  hypesthesia 
in  tlie  feet.  Among  sensory  irritative  symptoms,  Bramwell  and  I  have  de- 
scribed painful  cramps  of  the  calves.  Many  patients  complain  of  attacks  of 
rotary  vertigo  or  even  of  a  permanent  state  of  dizziness  of  little  intensity. 
Further,  the  disturbances  of  vegetative  function  which  have  been  occasionally 
Observed  in  Friedreich's  disease  should  be  considered.  These  are  profuse  saliva- 
tion, polyuria,  hyperidrosis,  oedema,  diabetes,  dyspnoea,  jerky  respiration,  dis- 
turbances in  swallowing,  late  beginning  of  the  menses.  The  sphincters  of  the 
bladder  anil  the  rectum  practically  always  functionate  correctly,  at  most,  slight 
degrees  of  urinary  incontinence  occur.  Impotence  is  never  present.  In  con- 
clusion the  rare  psychical  disturbances  must  still  he  considered.  One  patient 
studied  by  me  was  imbecile  to  a  high  degree,  at  the  same  time  vicious,  impulsive, 
and  given  to  coprolalia.  Other  authors  have  seen  a  combination  with  idiocy. 
Slight  defect  in  intellectual  development  occurs  somewhat  frequently;  usually, 
however,  the  thoroughly  normal   intelligence  of  such   patients  is  in  marked  con- 


Fig.  41. 
Friedreich's  Disease.     Typical  Foot  Deformity. 


126  LECTURE    VIII 

trast  to  the  weakminded  appearance  which  tlicy  make  in  consequence  of  their 
stumbling  speech  and  their  often  dull  expression  of  countenance. 

Pathological  Anatomy 

The  spinal  cord  in  Fried  nidi's  disease  is  thin  and  small  to  a  degree  observed 
in  no  other  disease;  also,  since  the  duration  of  the  disease  makes  no  difference 
in  this,  it  is  evidently  not  an  atrophy  but  a  hypoplasia  of  the  organ. 

We  consider  the  columnar  degenerations  which  we  find  in  the  spinal  cord  in 
Friedreich's  disease  as  consequences  of  an  arrest  of  development ;  these  regularly 
affect  the  posterior  columns,  the  direct  cerebellar  tracts,  and  the  lateral  pyrami- 
dal tracts,  sometimes  also  Grower's  columns  and  the  anterior  pyramidal  tracts. 
Fig.  42  shows  you  the  characteristic  picture  with  the  Weigcrt-Pal  stain  for  the 


'■3&*?r  v/!!"* 


Fig.  42. 
Alteration  of  the  Spinal  Cord   in   Friedreich's   Disease.      Weigert-Pal   Stain. 

medullary  sheath.  Only  very  exceptionally  have  these  typical  combined 
columnar  scleroses  been  seen  to  develop  in  a  well-formed  and  in  no  way  hypo- 
plastic spinal  cord  (Ed.  Mutter).  The  connection  of  the  ataxia  with  the  de- 
generation of  the  posterior  columns  and  the  spino-cerebellar  tracts  we  have 
already  brought  out.  Upon  comparing  the  anatomical  and  the  clinical  pictures, 
however,  the  fact  that  in  spite  of  extensive  alteration  of  the  posterior  columns 
so  little  sensory  defects  are  to  be  found  appears  paradoxical.  It  seems  as  if, 
in  system  diseases  which  affect  the  youthful  organism  the  nervous  conduction 
paths  so  organize  themselves,  that  by  a  roundabout  way  through  the  gray  sub- 
stance the  loss  of  the  posterior  columns  is  partially  compensated.  The  sub- 
stratum for  nystagmus,  choreic  unrest  and  disturbances  of  speech  is  anatomi- 
cally not  yet  made  clear.  Hypo-  or  areflexia  appears  to  correspond  to  the  loss 
of  function  of  the  fibers  of  the  posterior  roots.  The  degeneration  of  the  pyrami- 
dal tracts  usually  manifests  itself  only  by  Babimki's,  though  sometimes  also 
by  Oppenheim's,  reflex. 


THE    HEREDITARY    FAMILY    ATAXIAS  127 

Course  and  Prognosis 

The  beginning  of  the  disease,  in  the  great  majority  of  cases,  falls  between 
the  6th  and  the  loth  years  of  life.  An  earlier  commencement  (3  to  4>  years)  is, 
however,  not  exactly  rare,  neither  are  cases  which  present  their  first  symptoms 
at  from  15  to  20  years.  Unusual,  however,  are  the  "late  forms"  of  the  disease 
in  which  the  initial  symptoms  appear  in  the  third  decade  of  life.  As  to  the 
sequence  of  disease  symptoms,  they  usually  begin  with  ataxia  of  the  legs  and 
hyper-extension  of  the  great  toe;  next  the  patellar  reflexes  disappear.  The 
speech  disturbance  generally  is  not  long  delayed,  neither  is  involvement  of  the 
arms,  while  nystagmus  is  postponed  somewhat  longer.  Nevertheless,  within 
5  or  6  years  after  its  beginning,  the  disease  picture  is  usually  present  in  a 
typical  manner.  Further  progression  occurs  then,  as  a  rule,  much  more  slowly, 
so  that  the  patients  can  live  3  or  4  decades  longer,  until  some  intercurrent 
disease  (usually  broncho-pneumonia)  causes  death.  Remissions  are  rare,  more 
frequent  are  sudden  exacerbations  in  connection  with  intercurrent  acute  in- 
fections. 

Etiology  and  Pathogenesis 

In  spite  of  the  "erratic"  cases  which  not  so  rarely  come  under  observation, 
Friedreich's  disease  is  in  general  characterized  by  the  following  criteria,  as  an 
essentially  heredo-degenerative  disease,  corresponding  to  a  pathological  variety 
of  the  species.  It  attacks,  as  a  rule,  several  members  of  the  same  generation 
(  homologous  heredity)  and  occurs  in  these  mainly  at  about  the  same  age  (homo- 
chronous  heredity)  ;  it  is  not  to  be  referred  to  external  factors  during  intra-  or 
extra-uterine  life  (traumata,  intoxications,  infections);  in  such  factors  an  ex- 
citing cause  can  at  most  be  recognized.  The  injury  has  already  affected  the  germ 
(endogenic  basis);  and  finally  from  the  moment  of  its  beginning  it  is  marked 
by  unceasing  progression.  That  Friedreich's  disease  not  only  as  a  disease  of 
the  single  individual,  but  also  as  that  of  a  line  of  descendants,  manifests  a 
progressive  and  severe  degenerative  character,  is  shown  from  the  confirmed  fad 
that  from  generation  to  generation  the  age  at  which  it  begins,  becomes  lower 
and  lower.  Also  it  can  happen  that  the  disease  skips  several  generations  to 
appear  again  (by  atavistic  reversion),  which  is  denominated  latent  heredity. 
The  most  convincing  example  of  latent  heredity  is  furnished  by  a  genealogical 
(iii  prepared  by  h'onrad  Fret/,  which  refers  to  a  large  group  of  cases  of 
Friedreich's  disease  in  a  village  of  tin-  Swiss  Jura.  The  author  was  able,  by  re- 
ferring to  tie-  church  records,  to  recognize  the  common  descent  of  these  patients 
from  an  ancestor  of  the  sixteenth  century.  In  Hie  descendants  of  this  man,  dis- 
tributed among  six  collateral  lines,  the  disease,  however,  only  appeared  in  t  lu- 
ll th  or  12th  generation,  although  in  the  intervening  generations  consanguine 
marriages  with  tin-  loss  of  ancestors  and  a  history  of  heavy  drinking  was  often 
Doted. 

As  signs  of  congenital  defect  of  certain  parts  of  the  central  nervous  system 
mav  he  mentioned  the  frequent  accompaniment  of  Friedreich's  disease  by  the 
most  varied  congenita]  defective  conditions  and  malformations,  for  example,  by 
hypospadias,  facial  asvnnnetrv,  "mongoloid"  conformation  of  the  face,  anoma- 


128  LECTURE    VIII 

lies  of  the  central  canal  of  the  spinal  cord,  ectopy  of  the  gray  matter  of  the 
spinal  cord,  etc.  Or  the  combination  with  other  heredo-degenerative  diseases 
also  occurs ;  so,  for  example,  I  have  described  a  patient  with  hereditary  ataxia 
and  muscular  dystrophy  at  the  same  time,  and  Kollorits  has  seen  the  combination 
of  Friedreich's  disease  and  Huntington's  chorea.  Now  how  this  congenital  de- 
fective condition  ("abiotrophy"  according  to  Gowers)  leads  to  the  gradual 
degenerative  destruction  of  certain  tracts,  there  may  be  different  conceptions. 
Jendrdssik  writes:  "In  many  families  striking  peculiarities  occur.  Some  lose 
their  hair  prematurely,  in  others  the  pyramidal  tracts  degenerate."  Raymond 
has  spoken  of  "premature  senescence,"  Edinger  of  the  using  up,  I  of  the  wear 
and  tear  of  the  congenitally  defective  systems.  As  causes  for  the  first  appear- 
ance of  a  heredo-degenerative  disease  in  a  definite  family  line,  certain  "germ 
injuring"  or  "blastophthoric"  factors  cannot  infrequently  be  made  responsible. 
In  Friedreich's  disease  it  is  very  often  alcoholism  in  the  parents  (in  many  cases 
there  is  a  spontaneous  statement  that  the  affected  children  were  begotten  while 
the  father  was  in  a  drunken  condition!)  Further,  consanguinity  of  the  parents, 
advanced  age,  or  great  disproportion  in  their  ages.  As  exciting  causes  of  the 
disease  we  find  exceedingly  frequently  the  history  of  having  passed  through 
some  exhausting  disease  (variola,  typhoid  fever,  scarlatina,  measles,  pneumonia, 
pertussis,  meningitis,  influenza). 

Treatment 

Unfortunately  we  can  in  no  way  influence  the  disease  process  in  Friedreich's 
disease.  In  order  to  delay  its  course  as  much  as  we  can,  it  is  indicated  that  the 
patients  be  placed  as  soon  as  possible  under  the  best  hygienic  conditions  and 
under  continued  medical  care,  for  example,  best  in  an  institution.  In  every  case 
care  should  be  taken  that  they  get  plenty  of  bodily  rest,  best  lying  out  in  the 
fresh  air,  while  the  amount  of  daily  exercise  should  be  very  carefully  regulated 
so  that  overexertion,  which  is  under  all  circumstances  injurious,  is  avoided.  By 
careful  massage  it  should  be  endeavored  to  strengthen  the  muscles  still  capable 
of  function  and  to  effect  some  correcture  of  the  foot  deformity.  In  order  to 
relieve  the  ataxia  the  F 'renkel-Le yden  compensation  therapy  which  we  will  con- 
sider at  length  under  the  discussion  of  tabes  in  the  next  lectures,  should  be  tried 
in  every  case;  however,  I  have  only  once  obtained  a  decided  effect  which  was 
lasting.  From  time  to  time  strychnine  cures  (for  adults  daily  0.002  to 
0.005  (gr.  1/30  to  gr.  1/12)  subcutaneously,  or  4  times  a  day  5  to  10 
drops  of  tinct.  nucis  vomica?),  are  also  to  be  recommended.  In  order  to  prevent 
bed-sores,  in  bedridden  patients  there  should  be  the  most  thorough  cleansing 
and  care  of  the  whole  back,  as  well  as  proper  attention  to  position  in  bed. 

II.    Cerebellar  Heredo  Ataxia 

In  the  year  1893  Senator  called  attention  to  some  cases  of  hereditary 
ataxia  in  which,  along  with  the  spinal  lesion  which  we  have  described  (see 
page  125)  there  was  ^also  a  striking  diminution  in  size  of  the  cerebellum,  and 
in  the  same  year  P.  Marie  attempted  to  construct  from  these  atypical  cases 
a  disease  picture  to  which  he  gave  the  name  "heredo-ataxie  cerebelleuse,"  and 


THE    HEREDITARY    FAMILY    ATAXIAS  129 

which  was  to  bo  separated  both  clinically  and  anatomically  from  Friedreich's 
disease. 

According  to  Marie's  description,  the  marked  cases  present  the  picture  of 
a  disease  which  begins  after  the  twentieth  year  of  life  with  a  slowly  and  pro- 
gressively increasing  unsteadiness  in  walking  and  standing;  sometimes,  too, 
with  pains  in  the  loins  or  in  the  lower  extremities.  After  from  one  to  three 
years  the  ataxia  involves  the  arms  also,  while  at  about  the  same  time  dis- 
turbances of  speech  and  of  vision  become  evident.  Optic  atrophy  and  narrow- 
ing of  the  visual  field,  often  also  a  loss  of  the  pupillary  reaction  to  light,  are 
found.  The  patellar  reflexes  are  exaggerated,  or  at  least  of  normal  activity. 
Often  there  are  also  other  spastic  phenomena;  for  example,  ankle  clonus.  Fre- 
quently a  certain  mental  weakness  is  observed.  Nystagmus  and  disturbance 
of  speech  develop  as  in  spinal  hereditary  ataxia.  On  the  other  hand,  the 
deformities  of  foot  and  spine  so  characteristic  of  the  latter  are  absent.  Quite 
often  there  are  disturbances  of  sensibility,  less  frequently  those  of  swallowing 
and  of  the  bladder;  sometimes  there  is  hypertonia  of  the  muscles,  again  chorei- 
form movements.  This  presents  a  symptom  complex  differing  in  many  impor- 
tant points  from  Friedreich's  disease,  which  seems  to  justify  definite  separa- 
tion from  affections  resembling  it  in  the  disturbances  of  coordination  and 
occurrence  in  families ;  but  it  has  been  shown  that  the  boundary  line  cannot 
be  sharply  drawn  either  from  an  anatomical  or  from  a  clinical  point  of  view. 
As  to  the  post-mortem  findings,  it  suffices  to  point  out  that  Thomas  and  Roux 
in  one  case  which  corresponded  particularly  well  with  the  above  description  of 
Marie,  have  reported  a  negative  finding  as  regards  the  cerebellum,  with  hypo- 
plasia and  systematic  degeneration  of  the  spinal  cord,  and  that  I  found  in  a 
patient  who  presented  clinically  not  a  single  one  of  the  symptoms  character- 
istic for  Marie's  type,  the  most  profound  alterations  of  the  cerebellum  which 
have  ever  been  observed  in  the  hereditarily  ataxic.  The  weight  of  the  cere- 
bellum was  reduced  from  the  normal  figure  of  145.2  grms.  to  43.2  grms.  (see 
Figs.  43  and  44).  From  a  clinical  point  of  view,  we  know,  too,  that  Marie's 
type  of  hereditary  ataxia  can  occur  just  as  frequently  in  childhood  as  that  of 
Friedreich  in  adult  age;  that  in  it  there  may  be  intact  sensibility  and  an  intact 
Optic  nerve;  that  scoliosis  and  pes  cavus  with  dorsal  contracture  of  the  great 
toe  occur  now  and  then,  that  loss  of  the  pupillary  light  reflex  is  usually  absent, 
etc.  Exaggeration  of  the  reflexes  has  best  preserved  itself  as  a  clinical  cri- 
terion; hut  with  observing  that  the  patellar  reflex,  while  abnormally  lively  at 
the  start,  can  he  lost  during  the  course  of  the  affection,  this  last  distinction 
must  tail.  Also  hereditarily  ataxic  brothers  and  sisters  who,  while  presenting 
an  otherwise  identical  picture,  show  a  different  condition  of  I  he  tendon  re- 
flexes, are  not  at  all  rare.  Ileiiee,  we  can  recognize  cerebellar-Iieredo-at  a  \  ia 
only  as  a  relatively  characteristic  and  frequent  variety  of  the  true  Frii  dreieli's 
disease,  and  at  the  bedside  must  refrain  from  making  a  diagnosis  as  to  the  ana- 
tomical distribution  of  the  lesion  with  too  great  positiveness  (especially  as 
to  the  involvement   or  not  of  the  cerebellum),     Indeed,  I   have  been   able   to 

recognize  experimentally  that   in  the  dog  the  same  ataxic  symptom  < iples 

can  be  produced,  according  to  choice,  either  by  a  lesion  of  the  cerebellar  tracts 
and  spinal  cord  or  by  destruction  of  their  endings   in   lh<'   vermis  cerebelh. 


130 


LECTURE    VIII 


Fig.  43. 
Atrophic   Cerebellum   of  a   Patient   Aged    Forty   Years,   with   Hereditary   Ataxia. 


Fig.  44. 
Normal  Cerebellum  of  a  Man  Aged  Forty  Years. 


THE    HEREDITARY    FAMILY    ATAXIAS  131 

This  discovery  bridges  over  physiologically  also  the  clinically  as  well  as  ana- 
tomically already  obliterated  boundaries  between  the  Frcidrcich  and  the  Mark- 
types.  As  regards  etiology  and  therapy,  there  is  nothing  to  add  to  what 
has  already  been  stated  in  connection  with  the  discussion  of  Friedreich's 
disease. 

III.    Infantile  Progressive  Hypertrophic  Neuritis 

In  the  year  1890  Dejerine  described  a  "special  form  of  Friedreich's  disease 
with  muscular  atrophy  and  disturbances  of  sensibility"  which  since  then  has 
been  recognized  as  a  clinically  and  anatomically  quite  special  disease.  The 
affection  begins  in  childhood,  occurs  almost  always  in  children  of  the  same 
family,  and  has  the  following  symptoms:  Marked  ataxia  of  all  four  limbs; 
pes  equinovarus  excavatus ;  kyphoscoliosis ;  nystagmus — so  far  criteria  of 
Friedreich's  disease.  To  these  are  added,  however,  loss  of  the  pupillary  light 
reflex,  psychical  abnormalities,  marked  disturbances  of  sensibility,  and  marked 
muscular  atrophy  in  the  peripheral  parts  of  the  extremities,  neuralgiform  pains. 
Further,  a  very  great  hypertrophy  of  the  peripheral  nerve  trunks,  recognizable 
on  palpation,  is  found  in  these  patients.  This  depends,  as  post  mortems  have 
shown,  upon  a  very  great  increase  of  their  fine  and  coarse  connective  tissue, 
which  proliferated  in  great  circles,  surrounds  the  partly  preserved,  partly 
degenerated,  nerve  fibers.  The  nerve  trunks,  on  account  of  this,  are  thickened 
to  double  their  normal  size,  which  particularly  gives  to  the  cauda  equina  a 
characteristic  appearance.  In  the  spinal  cord  there  is  a  systematic  affection 
of  the  posterior  columns. 


LECTURE  IX 
Multiple    Sclerosis 

Gentlemen  :  The  disease  with  which  we  must  now  occupy  ourselves  and 
which  was  first  recognized  as  a  disease  picture  sm  generis  and  studied  by 
Vulpian  and  Charcot  (in  the  sixties  of  the  last  century)  must,  since  of  late 
our  attention  has  been  directed  to  its  rudimentary  and  atypical  forms,  be 
considered  as  a  comparatively  frequent  nervous  disease.  I  see  many  of  these 
cases  among  a  country  population  and  this  experience  agrees  with  that  of  other 
neurologists. 

Multiple  or  disseminated  sclerosis  of  the  brain  and  spinal  cord,  the  "sclerose 
en  plaques"  of  Charcot,  takes,  in  the  circle  of  nervous  diseases,  a  special  posi- 
tion, in  consequence  of  a  remarkable  antithesis  which  exists  between  its  clinical 
and  its  pathologico-anatomical  habitus.  Namely,  while  from  autopsy  to 
autopsy  the  topographical  distribution  of  the  disease  foci  varies  so  much  that 
the  preparations  from  the  brain  and  spinal  cord  in  two  cases  never  represent 
the  same  picture,  clinically  a  number  of  types  can  easily  be  separated  in  which 
the  individual  cases  may  present  a  remarkable  similarity. 

Nevertheless,  while  no  region  of  the  cerebro-spinal  apparatus  is  avoided  by 
the  lesions  of  multiple  sclerosis,  on  comparing  the  different  sections  it  is  clear 
that  the  sclerotic  foci  plainly  show  a  predilection  for  certain  regions;  in  the 
pons  they  affect  chiefly  the  basal  regions ;  in  the  cerebrum,  the  walls  of  the 
ventricles  and  the  corpus  callosum;  in  the  cerebellum,  the  medullary  substance; 
in  the  spinal  cord  their  preference  for  the  white  matter  is  so  evident  that 
ltibbert  in  his  time  affirmed  that,  the  borders  of  the  gray  matter  set  an  im- 
passable limit  to  the  extension  of  the  sclerotic  foci — a  view  which  to-day  is 
indeed  disproven. 

Pathological  Anatomy 

Fig.  45  shows  you  with  how  little  reference  to  the  structural  arrangements 
of  the  nervous  elements  the  sclerotic  foci  are  bounded.  Macroscopieally  these 
plaques,  whose  number  in  the  brain  and  spinal  cord  can  vary  from  a  few  to 
many  hundred,  present  a  reddish  or  yellowish  gray  appearance;  on  palpation 
they  give  a  feeling  of  increased  resistance.  Microscopically  the  sclerotic  tissue 
presents  itself  as  a  thick  felt-work  of  proliferated  glia  through  which  many 
nerve  fibers  still  pass.  These  last  have,  indeed,  mostly  lost  their  medullary 
sheaths,  but  the  naked  axis  cylinders  are  preserved ;  indeed,  a  new  formation 
of  axis  cylinders  appears  to  have  occurred.  This  persistence  of  the  conduct- 
ing elements  makes  the  absence  of  secondary  ascending  or  descending  degen- 
erations in  multiple  sclerosis  explainable;  it  speaks  for  the  fact  that  the  glia 

132 


MULTIPLE    SCLEROSIS 


133 


proliferation  is  the  primary,  while  the  destruction  of  the  medullary  sheath  is 
usually  a  secondary  phenomenon  (to  which,  indeed,  the  phagocytic  action  of  the 
glia  cells  contributes).  Also  the  ganglion  cells  in  the  foci  are  preserved;  on 
the  other  hand,  the  blood  vessels  are  usually  markedly  affected ;  they  are  thick- 
ened, infiltrated,  and  the  lymph  channels  of  the  adventitia  are  filled  with 
granular  cells. 

Etiology 

From  an  etiological  point  of  view,  multiple  sclerosis  presents  much  that 
is  puzzling.  The  most  widespread  opinion,  that  firmly  held  by  P.  Marie, 
attributes  to  it  an  infectious  basis.  Previous  typhoid,  variola,  measles,  scar- 
latina, have  frequently  been  brought  into  causal  connection  with  it.  The 
occurrence  of  great  epidemics  of  influenza  naturally  must  also  be  considered 
among  possible  etiological  factors. 
Less  frequently  puerperal  infec- 
tions, diphtheria,  erysipelas,  chol- 
era, dysentery,  and  malaria  have 
been  held  responsible.  Also  pre- 
vious syphilis  we  find  occasionally 
noted;  indeed,  one  of  the  cases 
reported  by  Thomas  and  Long  is 
remarkable  on  account  of  the  ana- 
tomically recognized  combination 
of  luetic  meningo-myelitis  and 
true  multiple  sclerosis. 

In  the  second  place,  poison- 
ings by  metals  have  been  brought 
forward  as  causal  factors,  chiefly 
by  Oppcnhcim.  In  these  cases 
tin  active  agent  has  usually  been 
lead,  but  some  cases  have  also 
been  attributed  to  intoxication  by 

mercury,  zinc  and  manganese.  Among  non-metallic  poisons,  carbonic  oxide 
must  be  mentioned,  since  in  a  number  of  observations  there  has  been  a  history 
of  poisoning  by  emanations  from  coal. 

Finally,  in  a  few  publications  the  disease  lias  been  referred  to  a  trauma  or 
to  a  violent   emotional   disturbance. 

Where  so  varied  a  list  of  factors  has  been  brought  into  etiological  relation 
with  one  and  the  same  disease,  we  are  quite  justified  in  seeing  in  these  factors 
rather  "provoking  agents"  than  direct  causes,  and  in  looking  deeper  for  I  lie 
actual  causes.  This  is  also  the  view  of  Strumpell,  who,  among  24  cases  ob- 
Krved  in  Erlangen,  was  never  able  to  prove  the  connection  with  previous 
infections  or  intoxications.  Apparently  multiple  sclerosis  is  not  an  exogenic, 
bui  in  endogenic  affection  dependent  upon  the  congenital  makeup  of  the 
nervous  system,  like  syringomyelia  to  be  described  later,  since  a  combination 
of  the  two  diseases  occurs.  This  agrees  with  the  experience  thai  multiple 
sclerosis,  as  a  rule,  is  a  disease  of  early  life;   its  beginning  is  usually  at    the 


Fig.   15. 
Sclerosis.     Section   through   the   Medulla, 
Weigert-Pal   Stain. 


134  LECTURE    IX 

end  of  the  second  or  the  beginning  of  the  third  decade.  In  children,  indeed, 
the  disease  is  quite  rare ;  here  one  cannot  be  too  cautious  about  the  diagnosis 
if  one  does  not  wish  to  incur  the  danger  of  being  contradicted  by  the  autopsy 
or  by  the  further  course  of  the  disease.  The  diagnosis  "infantile  multiple 
sclerosis,"  however,  was  confirmed,  for  example,  in  one  case  of  Eiclihorst,  which 
was  also  remarkable  in  that  the  mother  of  the  child  died  of  the  same  disease; 
however,  hereditary  occurrence  of  the  disease  is  extremely  rare.  Also,  the 
histo-pathological  picture  seems  to  me  to  speak  decidedly  against  an  inflam- 
matory basis  for  the  disease  as  assumed  by  different  authors;  much  rather,  it 
appears  to  me  as  the  correlate  of  a  congenital  tendency  to  perverse  growths 
somewhat  resembling  tumor  formations.  The  Russian  neurologist  Bossolymo 
goes  so  far  as  to  consider  multiple  sclerosis,  gliosis  and  glioma  of  the  central 
nervous  system  as  three  stages  of  intensity  of  the  same  process. 

Symptomatology 

When  now  we  come  to  sketch  the  clinical  s3-mptoms  we  will  confine  ourselves 
at  first  to  the  typical,  fully  developed  cases,  upon  which  Charcot  has  based 
his  classical  description. 

In  the  foreground  of  the  picture  stand  disturbances  of  motility,  above 
everything,  tremor.  This  presents  such  sharp  criteria  in  multiple  sclerosis 
that  in  spite  of  the  appearance  of  tremor  in  all  possible  nervous  diseases  it  can 
be  considered  the  most  characteristic  symptom  of  this  disease.  This  is  the 
so-called  "intention  tremor"  which  is  absent  during  rest  and  only  shows  itself 
on  voluntary  movements  (much  more  rarely  on  automatic,  reflex,  or  associated 
movements).  It  disappears  during  sleep;  under  the  influence  of  emotional 
disturbances  and  exertion  it  increases  and  its  oscillations  are  proportional  in 
their  range  to  the  extent  of  the  movements  carried  out ;  the  further  the  in- 
tended movement  goes,  the  more  rapid  the  oscillations  become,  but  in  spite  of 
this  they  preserve  a  certain  rhythm. 

In  a  classical  experiment  the  patient  is  solicited  to  carry  a  glass  of  water 
to  his  mouth.  The  moment  the  hand  begins  to  move  the  oscillations  com- 
mence ;  they  increase  in  extent  and  violence  the  nearer  the  glass  gets  to  the 
lips ;  it  strikes  clinking  against  the  teeth,  the  contents  are  spilled,  and  the 
tremor  ceases  only  after  the  glass  has  been  taken  away  from  the  patient.  If 
the  hand  of  the  patient  is  laid  upon  his  bed  and  he  is  made  to  bend  one  single 
finger,  this  isolated  intended  movement  suffices  to  set  the  whole  hand,  even  the 
entire  arm,  into  violent  oscillation.  The  tremor  of  multiple  sclerosis  is  a  massive 
one,  affecting  the  extremities  as  a  whole,  and  increases  in  intensity  toward  their 
roots.  In  like  manner  intention  tremor  can  be  provoked  in  the  lower  extremi- 
ties; the  head  and  neck  also  present  it;  as  long  as  the  patient  keeps  his  head  on 
the  pillow  they  are  quiet,  but  upon  raising  it  up  or  turning  it  sideways  the 
most  violent  nodding  and  rotating  oscillations  commence.  The  handwriting, 
even  in  the  early  stages  of  the  disease,  assumes  a  characteristic  appearance 
(as  in  Fig.  46). 

Not  only  the  impulse  necessary  to  carry  out  a  movement,  but  also  that 
needed  to  keep  a  certain  position,  sets  up   the  tremor.      So   the  body  of  the 


MULTIPLE    SCLEROSIS  135 

patient,  as  soon  as  he  attempts  to  hold  it  upright,  is  shaken  violently  up  and 
down,  or  forward  and  backward. 

In  the  arms,  as  in  the  legs,  the  tremor  may  be  first  unilateral,  or  at  least 
predominating  upon  one  side,  only  later  to  spread  to  the  opposite  side. 

As  to  the  material  basis  of  this  symptom,  there  are  various  hypotheses 
which  can  be  as  little  proved  as  disproved.  Charcot  considered  it  the  expres- 
sion of  the  leaping  over  of  the  stimulus  from  one  neuron  to  another  ( on 
account  of  the  loss  of  insulation  by  destruction  of  the  medullary  sheath). 
Later  authors  explain  the  intention  tremor  as  a  focal  symptom  produced  by 
sclerotic  plaques  in  the  pons,  in  the  corpora  quadrigemina,  in  the  thalamus, 
and  finally  in  the  cerebellum. 

Less  characteristic  is  the  disturbance  of  gait  in  patients  with  disseminated 
sclerosis.  Its  study  is  considerably  interfered  with  by  the  tremor  which  is 
at  the  same  time  set  up  on  walk- 
ing. In  general,  however,  three 
sorts  of  gait  abnormalities  can  be 
distinguished:  the  spastic,  the 
spastic  cerebellar,  and  the  purely 
cerebellar    gait. 

In  the  first,  with  the  beginning 
of  locomotion  there  is  tonic  rigid- 
ity of  both  lower  limbs ;  they  arc 
extended  at  all  joints,  and  besides 
this  held  in  adduction.  The  pa- 
tient balances  himself  on  the  tips 
of  his    toes,   as   we   have   described  I- IG-  4<i- 

under     spastic     paralysis.        This  .Multiple  Sclerosis.     Handwriting, 

form   is  not    rarely  observed;  still 

more  frequently,  however,  the  spastic  element  is  less  prominent  and  is  combined 
with  cerebellar-atactic  symptoms,  widening  the  supporting  surface  by  spreading 
the  legs,  swinging  the  feet,  and  deviating  from  side  to  side  from  the  straight 
line.  Much  more  rare,  on  the  other  hand,  is  the  third,  the  pure  cerebellar  type 
of  locomotion,  in  which  the  gait  is  staggering  and  uncertain;  the  feet  .seem  to 
stick  to  the  floor  and  forward  movement  is  carried  out  in  a  more  or  less 
zigzag  manner.  To  the  true  staggering  gait  of  the  drunken  man,  however, 
it  dors  not  usually  come,  because  sooner  or  later  spasticity  occurs.  Through 
this,  finally,  walking  becomes  impossible,  and  the  patient  is  condemned  to  bed 
on  account  of  true  spastic  paraplegia. 

Among  the  eye  muscle  disturbances  of  multiple  sclerosis,  nystagmus  lakes 
the  first  place.  In  the  early  stages  of  the  disease  it  must  he  sought  for  by 
making  the  patient  look  far  to  the  side  and  up  and  down.  It  is  a  dynamic 
nystagmus,  an  intention  nystagmus,  whose  intensity  increases  with  the  extent 
ol  the  movement.  Almost  always  lateral  nystagmus  is  observed,  more  rarely 
\ertie.d  nystagmus.  As  a  rarity,  rotary  nystagmus,  turning  the  eyes  like  a 
wheel,  has  been  described.  Do  not  forget  that  a  slighl  grade  of  nystagmus 
soinit imes  occurs  iii  healthy  people  by  strained  looking  to  one  side;  only  a 
definite  oscillation  of  the  eyes,  not  the  so-called  nystagmiform  contraction,  is 
to  be   relied    upon    for    He   diagnosis   of  multiple   sclerosis. 


136  LECTURE    IX 

Other  eye-muscle  disturbances  are  less  constant.  According  to  Dejerine 
and  Thomas,  they  only  occur  in  a  sixth  of  the  cases.  Usually  it  is  an  isolated 
paralysis  of  an  external  eye  muscle.  Most  frequently  affected  is  the  abducens, 
more  rarely  the  oculo-motorius.  Oppenheim  has  seen  three  cases  of  total 
ophthalmoplegia.  All  these  paralyses  come  on  slowly  and  by  degrees,  and 
can  disappear  later.  They  are  accompanied  by  diplopia  and  strabismus.  Ac- 
cording to  Kunn,  however,  the  strabismus  of  multiple  sclerosis  is  not  always 
the  expression  of  eye-muscle  paralysis,  but  the  result  of  sclerotic  foci  which 
interrupt  the  association  tracts  between  the  individual  eye  muscle  nuclei.  Myo- 
sis,  inequality  of  the  pupils,  weakness  of  accommodation,  occasionally  occur. 
More  frequently  hippus,  unrest  of  the  pupils,  abnormally  lively  pupillary 
reflexes,  are  observed,  which,  according  to  Parinaud,  are  to  be  considered  as 
the  homologues  of  intention  tremor  and  increase  of  reflexes  in  the  muscles 
of  the  body. 

These  last  affect  the  tendon  and  bone  reflexes  in  all  the  limbs,  particularly 
in  the  lower  extremities,  however,  and  are  generally  very  marked.  Usually 
the  whole  "spastic  symptom-complex,"  as  we  have  studied  it  in  Lecture  VIII, 
is  found  together  with  the  abnormal  reflexes  (Babinski,  Oppenheim,  Mendel- 
Bechterew)  ;  sometimes,  however,  the  ankle  clonus  cannot  be  obtained  on  ac- 
count of  the  foot  being  held  in  too  strong  contraction.  The  skin  reflexes, 
namely  those  from  the  abdominal  wall,  are,  however,  usually  diminished  or 
lost,  as  Eel.  Miillcr  has  specially  pointed  out.* 

The  tongue,  also,  and  the  speech  apparatus  are  the  seats  of  marked  dis- 
turbances of  motility.  When  the  patient  puts  out  his  tongue  it  is  noticed 
to  be  in  a  state  of  rhythmical  unrest;  it  is  alternately  protruded  and  drawn 
back,  in  rather  rapid  succession. 

The  speech  shows  marked  disturbances.  Only  slowed  at  the  beginning,  it 
takes  on  later  a  scanning  character.  Each  syllable  is  chopped  off  and  brought 
out  in  an  explosive  manner.  This  speech  has  very  appropriately  been  com- 
pared with  that  of  a  child  spelling.  The  individual  syllables  are  uttered  ex- 
plosively with  oscillations  of  the  head,  of  the  lower  jaw,  and  lips.  A  few 
authors  claim  to  have  observed  a  tremor  or  a  paresis  of  the  vocal  cords  in 
phonation.  Oppenheim  has  seen  this  sometimes  in  respiration,  which  becomes 
jerky  on  this  account.  In  spite  of  recent  attempts,  particularly  by  laryn- 
gologists  (for  instance,  Rethi,  in  Vienna),  to  analyze  the  disturbances  of  speech 
in  multiple  sclerosis,  a  satisfactory  explanation  of  its  method  of  production 
is  still  wanting. 

We  must  conclude  the  enumeration  of  motor  disturbances  with  description 
of  the  pareses  and  contractures  as  well  as  of  the  apoplectiform  attacks.  At 
the  start  the  gross  strength  in  arms  and  legs  is  well  preserved;  soon,  however, 
the  muscular  energy  diminishes  so  that  finally  there  may  be  actual  paralysis. 
Along  with  this,  even  in  the  early  stages  and  particularly  in  the  lower  extrem- 
ities, there  is  usually  a  condition  of  latent  contracture  which  is  expressed  in 

*  However,  abdominal  hyporeflexia  and  areflexia  are  only  to  be  considered  as  a  patholog- 
ical finding  in  young  persons  with  firm  abdominal  walls  which  contain  little  fat  and  in  the 
absence  of  disease  of  the  abdominal  organs!  Also  repeated  testing  with  stimuli  of  graduated 
intensity  is  necessary  before  the  abdominal  reflexes  are  put  down  as  weakened  or  lost. 


MULTIPLE    SCLEROSIS  137 

abnormal  resistance  to  passive  movements.  The  paretic  disturbances  are  often 
more  marked  on  one  side,  also  according  to  whether  they  affect  only  one  or  both 
limbs  of  the  affected  side,  they  present  the  monoplegic  or  the  hemiplegic  type. 
An  uncommon  symptom,  on  the  other  hand,  is  degenerative  muscular  atrophy, 
which  manifests  itself  by  qualitative  alterations  of  electric  irritability.  Com- 
plete reaction  of  degeneration,  so  far  as  I  know,  has  never  yet  been  recognized. 
Nevertheless,  such  cases  have  been  mistaken  for  amyotrophic  lateral  sclerosis 
by  no  less  a  person  than  Dejerine.  Actual  attacks  of  hemiplegia  occur  in 
only  a  small  part  of  the  cases.  These  are  accompanied  by  loss  of  consciousness 
and  leave  behind  a  one-sided  paralysis,  which,  however,  usually  disappears  very 
quickly,  in  a  few  hours  or  days.  I  have  frequently  seen  them  accompanied  by 
elevation  of  temperature.  Also  epileptiform  attacks  equally  accompanied  by 
a  rise  of  temperature  have  been  described  in  multiple  sclerosis. 

Bladder  troubles  are  not  at  all  infrequent,  though  in  my  experience  they 
occur  more  frequently  in  the  atypical  forms  than  in  cases  with  the  classical 
combination  of  symptoms;  they  are,  however,  usually  of  temporary  duration. 
They  are  generally  pollakiuria,  or  ischuria  paradoxa,  in  which,  when  the  bladder 
has  become  filled  to  a  certain  point,  the  urine  begins  to  leak  away  in  drops. 
More  rare  is  true  incontinence  with  permanent  dribbling  of  urine;  still  less 
frequent,  retention  of  urine  on  account  of  spasm  of  the  sphincters.  Disturb- 
ances of  the  rectal  and  of  the  sexual  functions  are  entirely  inconstant  and 
episodic.  When  impotence  or  priapism  are  complained  of,  they  are  usually 
temporary  conditions.  "Dissociated  disturbance  of  potence,"  in  which  erec- 
tions still  occur  but  ejaculation  and  orgasm  are  suspended,  deserve  special 
mention. 

Disturhances  of  sensibility  were  formerly  regarded  as  rarities;  they  are, 
however,  not  so  exceptional.  Frciind  first  directed  attention  to  them.  They 
are  chiefly  paresthesias,  tickling,  formication,  "going  to  sleep"  of  the  peripheral 
parts  of  the  extremities.  More  rarely  actual  pains,  stabbing,  boring,  girdle  or 
constriction  sensations.  Even  lancinating  pains,  like  those  in  tabes,  have  been 
pbserved  in  cases  certainly  multiple  sclerosis.  Neuralgias  also  occur.  As  cause 
of  Mich  pains  in  the  trigeminus  distribution,  in  one  case  a  focus  of  sclerosis 
situated  just  at  the  exit  of  the  nerve  has  been  described.  I  would  particularly 
point  out  the  arthralgias  which  are  not  at  all  rare,  and  which  may  lead  to 
confusion  with  articular  rheumatism  in  case  they  occur  at  the  start. 

Upon  objective  examination,  diminution  of  the  pain  and  temperature 
si'iim  ,  more  rarely  of  that  of  touch  and  vibration,  as  well  as  that  of  position 

sense,    is    found    reduced    in    some    patients.      The    topography    of    these    disturb- 

ances  is  extremely  inconstant,  ;ilso  they  can  totally  disappear  again.  Total 
anesthesia  scarcely  ever  occurs  as  an  actual  symptom  of  multiple  sclerosis. 
If  this  is  present  and,  still  more,  if  it  is  definitely  unilateral,  one  should  think 
of  hysteria  which  is  a  relatively  frequent  complication  of  this  disease.  Rarely, 
and  usually  as  an  episode,  hyperesthesia  is  complained  of. 

Upon  the  part   of  the  organs  of  sense  we  may   nexl   mention  changes  in 

the  optic  nerve,  which  are  to  lie  found,  indeed,  in  the  majority  of  cases.  They 
affect  now  only  one,  again  both  eyes,  and  produce  either  amaurosis  or  am- 
blyopia,  or   more   or   less    circumscribed   defects   in    the    visual    field.      These    last 


138  LECTURE    IX 

can  be  centrally  or  peripherally  located,  but  have  usually  a  quite  irregular 
outline.  Further,  the  visual  disturbances  are  exceedingly  variable  in  their 
intensity  and  can,  indeed,  entirely  disappear  again.  The  alterations  of  the 
eye  ground  can  present  themselves  under  very  varied  aspects  which  have  been 
studied,  among  others,  by  Unthoff,  Parinaud,  Brums  and  Stolting.  Only  excep- 
tionally has  the  papilla  the  appearance  of  optic  neuritis  (hyperemic,  somewhat 
prominent,  cloudy  with  dilated  vessels)  ;  usually  it  is,  on  the  contrary,  flat 
and  gray-white,  or  porcelain-white,  in  color.  The  circumstance  is  remarkable 
that  the  ophthalmoscopic  change  almost  never  affects  the  optic  nerve  in  toto, 
but  is  usually,  even  in  the  late  stages,  limited  to  one-half,  generally  the  tem- 
poral.* These  different  anomalies  contribute  to  give  the  patient  the  peculiar 
dull  look  with  which  Charcot  was  struck  when  he  expressed  himself,  "These 
patients  have  a  vacant  look." 

While  hearing,  as  a  rule,  remains  intact,  vertigo  of  vestibular  origin  belongs 
to  the  most  frequent  symptoms  of  multiple  sclerosis.  It  is  true  rotary  vertigo, 
sometimes  of  great  intensity,  so  that  the  patient  has  the  feeling  of  actually 
being  whirled  around,  and  on  this  account  falls  to  the  ground.  As  a  rule, 
this  vertigo  occurs  in  attacks  and  suddenly,  set  up  either  by  change  of  position 
of  the  head  (raising  up  in  bed,  looking  upward,  locomotion),  or  without  recog- 
nizable cause.  Only  very  exceptionally  it  comes  on,  like  Meniere's  disease, 
with  ringing  or  whistling  in  the  ears;  also  it  does  not,  as  a  rule,  lead  to 
vomiting.  In  some  cases,  specially  marked  by  decided  vertigo,  it  has  been  as- 
sumed that  there  were  sclerotic  foci  in  the  course  of  the  vestibular  fibers,  but  in 
only  a  very  few  have  there  been  any  positive  findings.  Though  Charcot  asserted 
that  vertigo  was  an  initial  symptom  in  three-fourths  of  all  the  cases  of  mul- 
tiple sclerosis,  from  my  personal  material  I  would  say  that  these  figures  are 
too  high ;  of  vertiginous  initial  symptoms  I  could  find  a  history  in  scarcely 
half  the  cases.  Taste  and  smell  are  usually  uninfluenced  in  disseminated 
sclerosis. 

Of  other  cerebral  disease  manifestations,  we  must  mention  headache  and 
psychical  alterations.  The  first  is  very  frequently  complained  of;  it  can  be 
like  migraine.  The  latter  often  occur  first  after  a  long  period  of  normal 
mental  condition;  finally,  however,  in  the  majority  of  cases.  The  patients 
are  either  silly  and  strikingly  euphoric,  or,  on  the  other  hand,  morose  and 
uninterested.  Also,  an  abnormal  lability  of  mood  which  manifests  itself  by 
sudden  change  between  laughter  and  tears  is  not  rare ;  along  with  this  there 
occurs  also  actual  compulsory  laughing  and  crying.  When  multiple  sclerosis 
begins  in  early  life,  it  usually  has  particularly  serious  results  psychically,  and 
puts  a  complete  stop  to  further  mental  development.  On  the  other  hand, 
where  hallucinations  and  delirium  have  been  described,  there  was  certainly  a 
combination  with  psychoses. 

Finally,  trophic  alterations  must  be  considered.  These  only  exceptionally 
occur;  epidermolysis,  dropsy  of  joints,  localized  oedema  and  sweating,  falling 

*  Since  normally  the  temporal  half  of  the  papilla  appears  paler  than  the  nasal  half  (the 
thinner  layer  of  nerve  fibers  allows  the  white  lamina  cribrosa  to  be  seen  more  plainly  there), 
in  doubtful  cases  it  is  always  advisable  to  get  the  opinion  of  an  experienced  ophthalmologist. 


MULTIPLE    SCLEROSIS  139 

out  of  the  hair,  brittleness  of  the  finger  nails,  have  been  described.  Only  in 
advanced  stages  of  the  disease,  when  the  patients  are  bedridden,  and  particu- 
larly in  cases  of  very  rapid  course,  is  there  a  marked  tendency  to  bedsores. 

Atypical  Forms  of  Multiple  Sclerosis 

The  classical  or  Charcot's  form  of  disseminated  sclerosis,  as  we  have  now 
become  acquainted  with  it,  is  marked  by  so  great  a  number  of  definite  symp- 
toms that  its  diagnosis  is  to  be  considered  a  very  easy  one — at  least,  as  easy 
as  that  of  tabes.  As  particularly  characteristic,  since  the  time  of  Charcot, 
the  ''symptomatological  triad" — intention  tremor,  nystagmus  and  scanning 
speech — have  been  brought  forward.  But  the  difficulty  in  recognizing  the 
disease  is  given  by  its  atypical  forms,  which  include  a  striking  majority  of  the 
cases,  according  to  my  experience,  at  least  90  per  cent.,  and  knowledge  of 
which  is  on  this  account  extremely  necessary.  Along  with  the  more  or  less 
rudimentary  forms  of  the  disease  in  which  different  cardinal  symptoms  are 
absent  (besides  Charcot's  triad,  I  would  add  to  these  last  also  spasticity  of  the 
legs  and  loss  of  the  abdominal  skin  reflexes*),  along  with  these  "formes 
frustes"  there  are  still  some  atypical  forms  distinguished  by  characteristic 
clinical  pictures  of  which  I  will  only  describe  to  you  the  four  most  important. 

(a)  The  Bulbar  Form. — In  this  difficulty  in  swallowing  and  chewing,  dis- 
turbances of  articulation  and  phonation,  stand  in  the  foreground,  glycosuria, 
rapidity  of  the  pulse,  and  asphyxic  conditions  occur.  When  one  considers 
how  often  upon  autopsy  large  sclerotic  areas  are  found  in  the  medulla,  it  appears 
paradoxical  that  these  clinical  disturbances  are  really  great  rarities  (see  Fig. 
45,  page  132). 

(I>)  The  Hemiparetic  Form. — In  this  form  spastic  paresis  and  loss  of  the 
abdominal  reflexes  are  present  only  on  one  side;  with  these  intention  tremor  may 
DCCUr  on  one  side  only.  Here  and  there  disturbances  of  sensibility  are  found 
upon  the  opposite  .side  in  such  cases,  also  the  Browrb-Sequard  symptom-complex 
of  half-sided  spina!  cord  lesions.  In  conditions  of  this  kind  a  positive  diagnosis, 
indeed,  can  scarcely  lie  made  before  the  autopsy,  but  with  very  slow  and  inter- 
mittent progress  of  thai  symptom  one  should  not  forget  to  think  of  multiple 
Sclerosis.     In  one  case  of  Oppcnhcim  this  diagnosis  could  be  confirmed. 

(i  )  Paraplegic  Form. — In  contrast  to  the  two  already  related,  this  atyp- 
ical form  is  extremely  frequently  observed.  In  the  initial  stages  the  picture 
of  spastic  spina]  paralysis  may  lie  present;  in  characteristic  form,  however, 
ii  -mi 1 1  \  loss  of  the  abdominal  skin  reflexes,  changes  in  the  optic  nerve,  or  slight 
intention  tremor  in  the  upper  extremities  may  direct  attention  in  the  right 
direction.  Slight  disturbances  of  sensibility  and  of  the  bladder  function  occur 
in  an  appreciable  number  of  these  rases.  In  the  further  course  very  severe 
spaslir  conditions  may  develop:  either  only  the  lees,  or  all  four  extremities 
Income  eont  raet  ured  little  bv  little,  the  arms  in  flexion,  tin'  legs  usually  in 
extension,  but  often  likewise  in  flexion.  Th<-  tendon  reflexes  are  exaggerated 
in  He    highest  degree,  and  on  testine-  them  marked  clonus  is  produced.     Later 


'  Willi  Hie  exceptions  mentioned  in  note  ""   page   135. 


140  LECTURE    IX 

permanent  organic  contractions  of  the  tendons  and  muscles  can  occur  (I  once 
saw  the  knees  gradually  drawn  up  to  the  chin  on  this  account),  when  the 
reflexes  can  no  longer  be  obtained. 

(cl)  The  Amyotrophic  Form. — I  have  already  cursorily  mentioned  this,  and 
indicated  that  it  can  imitate  amyotrophic  lateral  sclerosis.  There  is  atrophic 
paralysis  of  the  muscles  of  the  trunk  and  limbs,  usually  with  complete  reaction 
of  degeneration  and  permanent  contractures  in  the  paralyzed  region,  great 
increase  of  the  reflexes  and  bulbar  symptoms. 

Differential  Diagnosis 

The  special  peculiarity  of  multiple  sclerosis,  in  beginning  often  with  rudi- 
mentary and  atypical  symptoms  not  infrequently  leads  to  its  confusion  with 
other  nervous  diseases.  In  the  early  stages,  when  the  skin  reflexes  are  normal 
and  there  is  yet  no  spasticity  of  definitely  organic  character  (that  is,  no 
Babinski  or  Oppcnhcim  reflex  or  ankle  clonus,  etc.),  a  wrong  diagnosis  of 
hysteria  is  sometimes  made  even  by  experienced  observers,  a  mistake  which 
is  all  the  more  frequent  since  the  combination  of  both  diseases  is,  as  I  have 
already  remarked,  not  at  all  infrequent.  In  such  cases  it  is  well  to  be  very 
cautious  and  to  put  off  making  a  diagnosis  and  prognosis  until  the  further 
course  of  the  disease  has  been  observed.  In  those  rudimentary  cases  where 
tremor  is  the  only  symptom  (they  are  exceedingly  rare)  a  consideration  of 
the  typical  character  of  this  tremor  will  make  a  confusion  with  chorea  or  paral- 
ysis agitans  impossible.  A  differential  diagnosis  between  this  and  mercurial 
tremor  may  be  more  difficult;  this  is  often  an  intention  tremor,  which,  how- 
ever, does  not  cease  entirely  during  rest.  In  the  course,  or  as  a  consequence, 
of  infectious  diseases,  disseminated  myeloencephalitis,  whose  symptomatology 
presents  a  great  similarity  to  that  of  multiple  sclerosis,  may  occur.  This  runs 
a  course  with  elevation  of  temperature,  and  is  usually  characterized  by  some 
focal  symptoms  which  point  to  large  inflammatory  foci  in  the  brain  axis ;  for 
example,  an  alternating  hemiplegia  with  paralysis  of  the  face  on  one  side  and 
of  the  extremities  on  the  other.  Acute  onset  does  not  suffice  to  exclude  mul- 
tiple sclerosis ;  this  also,  as  we  will  soon  see,  may  commence  acutely.  In  many 
cases  only  the  further  course  will  clear  up  the  diagnosis.  Cerebro-spinal  syph- 
ilis can  also  simulate  multiple  sclerosis.  Lumbar  puncture  is  very  valuable 
here  when  syphilitic  infection  has  not  been  definitely  shown.  According  to 
Long  and  others,  in  multiple  sclerosis  there  is  no  lymphocytosis,  or  only  a 
minimal  one  is  found. 

In  general,  also,  the  Wassermann  complement-fixation  reaction  is  a  good 
differential  guide.  However,  I  once  saw  a  positive  result  from  this  in  a  case 
which  proved  on  autopsy  to  be  certainly  multiple  sclerosis,  although  there 
was  neither  a  history  nor  any  objective  signs  signifying  the  assumption  of 
previous  syphilis.  (It  was  in  a  virgin.)  In  the  picture  of  progressive  paral- 
ysis, also,  spastic  paresis  can  figure,  and  this  also  shows  tremor,  apoplecti- 
form attacks,  and  speech  disturbances.  Here,  however,  the  tremor  is  rapid 
and  fine  and  affects  chiefly  the  ends  of  the  extremities ;  the  speech,  too,  is  not 
scanning  and   chopped   off,  but   hesitating  and   drawling.      Finally,   also,   the 


MULTIPLE    SCLEROSIS  141 

psychical  disturbances  are  of  quite  definite  character.  It  is  to  be  noted  that 
in  a  number  of  cases  the  combination  of  progressive  paralysis  with  multiple 
sclerosis  has  been  observed  (for  example,  by  Schultz).  Those  rare  cases  of 
disseminated  sclerosis  which  begin  in  an  apoplectiform  manner  can  present 
difficulty,  since  post  hemiplegic  tremor  after  apoplexy  can  exceptionally  have 
a  typical  intentional  character.  In  such  cases  one  must  often  await  further 
developments  before  coming  to  a  conclusion.  Nevertheless,  in  suddenly  or 
gradually  occurring  hemiplegias  in  young  persons  in  whom  alcoholism,  brain 
tumor,  syphilis,  heart  disease  and  hysteria  can  be  excluded,  the  possibility  of 
multiple  sclerosis  should  not  be  lost  sight  of.  Many  points  in  common  are 
shown  by  the  symptomatology  of  multiple  sclerosis  on  the  one  hand,  and  of 
the  hereditary  ataxias  on  the  other,  namely,  nystagmus,  scanning  speech  and 
cerebellar  atactic  phenomena.  However,  there  can  be  no  confusion  with  the 
spinal  variety  of  Friedreich's  disease,  since  it  is  characterized  by  loss  of  tendon 
reflexes,  by  scoliosis,  and  by  the  foot  deformity ;  besides  the  tremor  combined 
with  ataxia  peculiar  to  it  has  entirely  a  different  character  from  that  of  multiple 
sclerosis  (see  the  previous  lecture).  Rather  is  there  a  possibility  of  confusing 
multiple  sclerosis  with  Marie's  cerebellar  heredo-ataxia,  since  in  this  the  reflexes 
are  exaggerated,  scoliosis  and  foot  deformities  are  absent,  and  the  tremor 
may  have  a  definitely  intentional  character;  further,  changes  in  the  optic 
nerve  occur.  Here  family  character  would  speak  for  heredo-ataxia;  besides, 
in  this  last  there  is  usually  beginning  with  purely  cerebellar  symptoms.  Brain 
tumors  sometimes  produce  clinical  pictures  similar  to  multiple  sclerosis,  as 
Hruiis  ami  Xonne  have  shown.  The  differential  diagnosis  can  be  greatly 
involved,  when  on  account  of  complicating  hydrops  ventriculorum  the  multiple 
sclerosis  is  accompanied  by  symptoms  of  brain  pressure  (continued  headache, 
oedema  of  the  papilla,  etc.).  In  general,  however,  these  last  are  not  so  in- 
tense, and  particularly  not  so  progressive,  as  in  intracranial  tumors.  Besides, 
these  are  exceptionally  rare  cases.  Again,  the  differentiation  between  tumor  of 
the  spinal  cord  and  multiple  sclerosis  is  sometimes  not  easy.  I  intend  to 
consider  this  practically  important  point  in  a  later  lecture  when  describing 
tumors  of  the  spinal  cord.  Finally,  Westphal's  "pseudo-sclerosis,"  a  rare  ami 
not  yet  sufficiently  explained  disease,  in  which,  anatomically,  the  findings  have 
been  negative  or  only  a  very  inconsiderable  degree  of  diffuse  glia  proliferation, 
must  lie  mentioned.  The  clinical  picture  has  great  analogies  with  that  of 
multiple  sclerosis;  still,  on  the  one  hand  nystagmus  and  optic  nerve  changes 
are  regularly  wanting,  and  on  the  other,  in  pseudo-sclerosis,  along  with  the 
nearly  constant  occurrence  of  epileptiform  attacks,  there  are  other  symptoms 
ffhich  do  not  belong  to  true  sclerosis;  namely,  deep  apathy  and  dementia, 
delirium,  outbreaks  of  violence  and  a  peculiar  slowing  of  the  movements  of 
the  lace  and  eye  muscles.  Here,  also,  upon  a  proper  estimation  of  the  symp- 
toms, mistakes  can  be  avoided  all  the  more,  since  pseudo-sclerosis  is  a  disease 
of  childhood,  while  multiple  sclerosis,  as  has  already  been  indicated,  almost 
never  occurs  in  children. 


142  LECTURE    IX 

Course  and  Prognosis 

We  can  distinguish  three  methods  of  beginning  of  multiple  sclerosis.  It 
can  begin  insidiously,  and  this  is  most  frequent,  and  usually  then  announces 
itself  by  "outpost  symptoms,"  as  Oppenheim's  appropriate  expression  has  it, 
for  example,  by  disturbances  of  the  eye  muscles  with  double  vision  or  by 
changes  in  the  optic  nerve  (often  wrongly  considered  as  retro-bulbar  neuritis), 
or  by  bladder  troubles;  to  these  are  usually  added  spastic  symptoms  in  the  legs, 
which  little  by  little  increase  in  intensity  ;  less  frequent  is  a  rapid  (acute  or  sub- 
acute) beginning,  usually  introduced  by  severe  vertigo,  still  more  rarely  sudden 
brutal  onset,  with  an  apoplectiform  or  epileptiform  attack.  The  disease  itself 
runs  a  more  or  less  chronic  course,  either  progressively  or  in  exacerbations. 
In  the  first  case  the  disease  usually  leads  to  death  in  from  5  to  20  years. 
Proximal  causes  of  death  are:  intercurrent  diseases  or  the  occurrence  of  bulbar 
disturbances,  cachexia  with  bedsores,  or  an  apoplectic  attack.  There  are  cases 
described  as  acute  multiple  sclerosis,  which  in  from  3  months  to  2  years  de- 
velop rapidly  the  most  severe  symptoms  and  end  fatally.  Probably  these,  or  at 
least  part  of  them,  are  instances  of  another  disease,  namely  the  post  infectious 
disseminated  myeloencephalitis  mentioned  in  the  discussion  of  differential  diag- 
nosis. When  remissions  occur  they  can  last  for  years ;  in  one  of  my  cases 
affecting  a  50-year-old  lady,  the  history  mentioned  that  at  the  beginning  of  her 
third  decade  of  life  she  had  suffered  from  a  temporary  spastic  paraplegia 
with  tremor;  a  nearly  thirty-year  remission  must  hence  be  assumed  in  this  case. 
The  possibility  of  recovery,  or  at  least  of  a  definite  standstill,  of  the  disease 
is  affirmed  by  a  few  authors,  but  these  reports  must  unfortunately  be  received 
with  great  reserve,  as  the  above  case  with  a  recurrence  after  27  years  shows. 
Important  from  the  point  of  view  of  prophylaxis  is  the  condition  that  new 
exacerbations  are  often  started  up  by  overexertion,  getting  chilled,  infectious 
diseases,  the  puerperium,  etc. 

Treatment 

After  entering  upon  hospital  treatment  considerable  improvement  (which 
is  mainly  to  be  attributed  to  the  physical  and  psychical  rest  which  the  patient 
gets)  is  often  observed.  Also  in  patients  cared  for  at  home,  the  chief  weight 
is  to  be  laid  upon  the  keeping  away  of  causes  for  overexertion  and  emotions. 
In  this  connection  it  is  to  be  remembered  that  these  patients  often  have  a 
strong  libido,  whose  gratification  or  even  excitation  is  to  be  suppressed  as  far 
as  possible.  This  point  must  be  considered  also  in  the  choice  of  nurses ;  an 
endeavor  must  be  made  to  strengthen  and  build  up  the  patient.  Warm  baths  are 
just  as  injurious  as  cold  water  treatment.  The  same  remarks  apply  to  strong 
electric  applications.  Of  all  drugs,  only  arsenic  seems  to  have  any  active  effect 
in  the  way  of  favoring  remissions.  I  give  it  either  in  the  form  of  Folder's 
solution  or  as  the  Asiatic  pill  (raising  it  as  rapidly  as  possible  to  considerable 
doses,  7  drops  of  Fowler's  solution  or  0.005  (gr.  %s)  of  acid,  arsenios,  t.i.d.), 
eventually  also  in  combination  with  quinine  or  iron,  or,  however,  if  there  is  no 
effect  after  3  weeks  from  this  medication,  subcutaneously  as  sodium  cacodylate, 
1  ampoule  containing  0.05  (gr.  %),  later  0.1  to  0.15  (gr.  1^4  to  gr.  2). 


LECTURE   X 
A.    Spinal  Gliosis  and  Syringomyelia 

The  two  disease  conditions  of  which  we  will  now  give  a  common  description 
cannot  be  separated  from  one  another  either  symptomatologically  or  upon  a 
basis  of  their  pathologico-anatomical  substratum,  and  their  pathogenesis;  since 
spinal  gliosis  not  only  is  a  condition  regularly  antecedent  to  the  occurrence 
of  the  cavity  formation  which  we  call  "syringomyelia,"  but  both  conditions  are 
usually  found  alongside  of  one  another  in  the  spinal  cord  of  one  and  the 
same  patient. 

Pathological  Anatomy 

A  typical  syringomyelic  spinal  cord  can  macroscopically  closely  resemble 
a  tube,  justifying  the  characteristic  appellation  introduced  by  Ollivier  cV Angers. 

The  former  view  that  it  was  the  product  of  an  ectasy  and  a  hydrops  of 
the  centra]  canal  must  be  given  up,  since  the  entire  independence  of  the  cavity 
formation  from  the  spinal  canal  can  often  be  made  out.  From  other  cases  there 
is  evidence  that  the  confluence  of  the  ependymal  canal  and  the  syringomyelic 
cavity  have  occurred  secondarily  only.  This  last  usually  begins  at  the  pos- 
terior gray  commissure  and  extends  more  or  less  irregularly  in  the  cross  section 
into  the  gray  substance  of  the  posterior  and  anterior  horns.  In  a  longitudinal 
direction  it  extends  from  the  cervical  region  (at  which  level  the  cavity  formation 
usually  begins)  in  the  majority  of  cases,  getting  smaller  continually,  into  the 
middle  or  lower  thoracic  region.  There  are,  however,  instances  in  which  if  has 
extended  deep  into  the  lumbar  region,  indeed,  into  the  conus  terminalis.  On 
tin-  other  hand  the  process  can  ascend  until  the  cavity  opens  into  the  4th  ven- 
tricle of  the  brain  axis  and  involves  an  extensive  part  of  the  medulla,  when 
"syringo-bulbia"  can  properly  be  spoken  of. 

Microscopical  examination  now  furnishes  information  as  to  why  these  cavi- 
ties occur.  Their  walls  consist  mainly  of  a  thick  zone  of  firm,  greatly  pro- 
liferated, neuroglia  rich  in  cells"-*— that  is  of  the  same  tissue  as  the  neoplasms 
which  develop  from  the  supporting  substance  of  the  nerve  centers,  the  gliomata. 
While  glioma,  however,  spreads  from  its  point  of  origin  diffusely  in  every  di- 
fection  without  its  growth  following  any  definite  path  determined  by  anatomical 
relations  so  far  as  can  be  recognized,  the  process  upon  a  basis  of  which  syringo- 
myelia occurs  "central  gliosis  of  the  spinal  cord"-  is  characterized  by  more 
or  less  cylindrical  or  prismatic  outline,  by  excessively  slow  growth  and  tendency 
to  quiescence  for  long  periods,  and  by  exceedingly  sharp  separation  from  the 
healthy   tissues.      Peculiar  to   it,   further,   is   its   tendency   to  break   down   and   to 

148 


144 


LECTURE    X 


form  cavities ;  still,  cases  occur  in  which  the  last  are  absent  or  only  rudimentary, 
and  in  which  disease-pictures,  not  to  be  separated  clinically  from  syringomyelia, 
depend  upon  a  substitution  of  solid  masses  of  glia  for  central  portions  of  the 
spinal  cord.  Above  and  below,  too,  a  portion  of  solid  gliosis  is  often  joined  to 
the  glia  tube  of  syringomyelia.  Hence,  when  we  in  the  following,  for  the  sake 
of  simplicity,  speak  always  of  syringomyelia,  it  must  be  understood  in  advance 
that  our  remarks  apply  throughout  to  both  conditions  which  can  be  separated 
neither  clinically  nor  anatomically. 

The  syringomyelic  cavities  have  a  lining  of  ciliated  cylindrical  cells  only  in 
front  where  they  have  become  united  with  the  central  canal,  some  of  the  ependy- 


Fig.  47. 
Central  Gliosis   Passing  Over   Into   Syringomyelia.     Weigert-Pal   Stain. 

mal  lining  of  which  has  remained.  Finally,  where  the  gliomatous  masses  have 
overstepped  the  boundaries  of  the  gray  substance  into  the  white  columns,  as- 
cending or  descending  degeneration  of  the  long  fiber  tracts,  for  example,  of  the 
posterior  columns  or  of  the  pyramidal  tracts  results. 


Etiology 

Although  a  varied  line  of  exciting  causes  have  been  held  responsible  for  the 
outbreak  of  syringomyelia — that  is,  for  its  first  clinically  determined  symptoms 
— there  can  be  no  doubt  that  it  is  at  the  base  a  congenital  process,  namely  that 
the  affected  individual,  on  account  of  a  congenitally  defective  structure  of  the 
spinal  cord,  is  condemned  to  defective  growth  and  degeneration  of  the  central 
glia  leading  to  disturbances,  first  unnoticeable,  but  gradually  becoming  more 
marked.  The  disease  affects  men  about  three  times  as  frequently  as  women ;  it 
shows  peculiar  variations  in  its  geographical  distribution;  for  example,  while 
quite  frequent  in  Vienna,  in  our  neighborhood  (northwest  Switzerland,  southern 
Baden  and  upper  Alsace)  it  is  excessively  rare. 


SPINAL    GLIOSIS    AND    SYRINGOMYELIA  145 

Symptomatology 

The  most  characteristic  symptom  of  syringomyelia  is  the  so-called  "disso- 
ciated anesthesia,"  that  is,  loss  of  pain  and  temperature  sense  with  intact  touch 
sense  and  deep  sensibility.  This  peculiar  disturbance,  first  thoroughly  studied 
by  K abler  and  Schultze,  has  been  denominated  the  "posterior  horn  type  of 
sensory  anomaly."  As  to  its  anatomico-physiological  basis  we  have  quite  accu- 
rate knowledge.  Certain  definite  short  fibers  from  the  posterior  roots  end  in  the 
posterior  horn  of  the  spinal  cord  on  the  same  side.  From  this  point  the  impulses 
transmitted  centripetally  by  these  are  conducted  farther  through  the  gray  sub- 
stance to  the  lateral  columns  of  the  opposite  side  and  through  them  to  the  optic 
thalamus  and  the  cerebrum  (by  way  of  the  "tractus  spino-thalamicus"). 

The  senses  of  pain  and  temperature  are  conducted  exclusively  by  these  paths, 
hence  in  destruction  of  the  posterior  horns  or  of  the  central  gray  matter 
(through  which  the  "tractus  spino-thalamicus"  passes)  the  perception  of  these 
sensations  is  lost,  while  to  touch  sense  and  deep  sensibility,  still  other  paths  re- 
main open  (the  posterior  columns,  etc.,  see  Fig.  50). 

In  typical  cases  of  syringomyelia  corresponding  to  the  predominant  distri- 
bution of  the  pathologico-anatomical  process  in  the  upper  segments  of  the 
spinal  cord,  the  dissociated  loss  of  sensation  is  found  in  the  upper  limbs  and  on 
the  chest  and  trunk.  The  patients  themselves  often  become  aware  of  these 
anomalies,  since  they  are  struck  by  the  absolute  painlessness  of  accidental  burns 
or  cuts  and  the  absence  of  feeling  of  cold  in  winter.  It  is  remarkable  also  that 
in  taking  the  history  of  individuals  in  whom  syringomyelia  has  only  been  diag- 
nosed in  the  third  or  fourth  decade  of  life  through  the  onset  of  other  symptoms, 
we  sometimes  meet  with  the  statement  that  even  as  children  they  had  been  re- 
markable through  experiencing  no  pain  from  wounds  and  burns. 

If  the  dissociated  loss  of  sensation  presents  nothing  alarming  to  the  pa- 
tient or  his  surrounders,  on  which  account  the  physician  is  generally  not  called 
into  counsel,  the  two  other  so-called  "cardinal  symptoms"  of  syringomyelia 
present  the  more  striking  and  disturbing  anomalies;  namely,  the  progressive 
muscular  wasting  usually  limited  to  the  upper  extremities  and  the  shoulders  and 
the  manifold  tropho-vasomotor  disturbances  equally  affecting  by  preference 
these  parts. 

The  muscular  atrophy  is  usually  for  a  long  time  unilateral  or,  at  least,  much 
more  marked  on  one  side;  only  in  the  later  course  of  the  disease  does  it  become 
more  or  less  symmetrical.  It  begins  almost  always  in  the  small  muscles  of  the 
band,  especially  in  those  of  the  first  intermetacarpal  space  and  of  the  thenar  and 
hypothenar  regions.  By  degrees  deformities  which  we  have  fully  described  in 
Lecture  VI  under  Spina]  Muscular  Atrophy,  of  the  type  Artin-Diicliiiinf.  occur. 
The  "leaping  over"  of  the  atrophy  from  the  hand  to  the  shoulder  muscles  OC- 
eui's  less  frequently  in  syringomyelia  than  in  tin-  Aran-Duch-ervnedisca.se;  some- 
what more  frequently  regular  ascent,  in  which  first  the  forearm,  and  then  the 
arm  muscles  are  attacked,  is  observed.  In  the  forearm  it  is  remarkable  that  the 
extensor  muscles  long  remain  intact;  the  preponderance  that  they  gain  over 
the  atrophied  muscles  of  the  median  and  ulnar  distribution  sometimes  results  in 
a  permanent  contracture  of  the  hand  in  a  position  of  hyperextension  which  it 


146  LECTURE    X 

has  been  attempted  to  characterize,  not  very  happily,  as  the  '"preacher's  hand." 
Much  less  frequently  we  meet  syringomyelic  muscular  atrophy  in  the  muscles  of 
the  trunk  and  leg;  these  have  a  somewhat  variable  manner  of  appearance  and 
can  give  rise  to  different  anomalies  of  position  (for  example,  pes  equinovarus. 
pes  calcaneus,  clawfoot,  etc.).  In  the  neck  the  sternocleidomastoid,  the  scaleni 
and  the  trapezii  are  affected  by  the  muscular  wasting.  The  affected  muscles 
almost  always  atrophy,  not  as  a  whole,  but  after  the  "fascicular"  type,  that  is, 
one  bundle  after  another.  The  proximity  of  degenerating  and  intact  muscular 
fibers  brings  it  about  that,  like  in  spinal  progressive  muscular  atrophy,  re- 
action of  degeneration  is  often  very  difficult  to  recognize,  and  then  is  only 
partial.  Fibrillary  contractions  are  usually  plainly  manifest;  they  are  found 
most  easily  in  the  deltoid. 

The  vasomotor  trophic  anomalies  affect,  above  everything  else,  the  skin  and 
its  derivatives — hair,  nails,  etc.  A  firm  oedema  of  the  back  of  the  hand,  cold- 
ness and  livid  coloration  cf  the  integuments  (Marhwaco's  '"succulent  hand") 
is  frequent.  A  similar  thing  is  sometimes  observed  in  the  foot.  Further,  ab- 
normal dryness,  scaling,  and  fissuring  of  the  skin,  tendency  to  herpetic  erup- 
tions, separation  of  the  epidermis  and  superficial  ulcerations  are  observed.  In 
the  origin  of  these  last  phenomena,  a  considerable  role  should  be  attributed  to 
the  fact  that  the  patients  injure  their  analgesic  and  thermo-ancsthetic  hands 
by  striking  them  against  things,  rubbing  their  wounds,  handling  hot  or  very 
cold  objects.  There  is  also  a  more  or  less  extended  atrophy  of  the  skin  with 
loss  of  its  normal  superficial  outline,  the  so-called  "glossy  skin."  I  would  men- 
tion further  hyperkeratosis  of  the  palms  and  soles,  brittleness  or  falling  out  of 
the  finger  nails,  loss  of  the  skin  hairs.  The  trophic  disturbances  affecting  the 
subcutaneous  connective  tissue  and  the  bones  of  the  phalanges  are  particularly 
severe.  Their  points  of  origin  are  usually  areas  of  suppuration  which,  starting 
in  the  fissures  of  the  cracked  skin,  whose  circulation  also  is  much  impaired,  do 
not  tend  to  heal  normally,  but  continually  to  grow  deeper.  Their  entirely  pain- 
less course  justifies  the  expression  "panaris  analgesique"  used  by  Morvan  for 
such  cases.  This  can  go  on  to  severe  mutilations  as  in  leprosy,  to  the  loss  of  the 
ends  of,  or  of  whole,  fingers.  Finally,  in  syringomyelia  "'osteoarthropathies"  in 
the  extremities,  also  in  the  vertebral  column,  occur,  and  indeed,  quite  frequently 
(according  to  Schlesinger  in  30  per  cent,  of  the  cases).  These  affect,  usually, 
the  shoulder  or  the  elbow.  Atrophic  and  hypertrophic  changes  in  the  head  of 
the  bone,  erosion  of  the  socket,  loosening  of  the  ligaments,  great  intracapsular 
effusions,  etc.,  are  found. 

We  leave  now  the  so-called  "cardinal  symptoms''  to  turn  our  attention  to 
the  less  frequent  manifestations  of  syringomyelia.  Most  important  among 
these  last  are  the  oculo-pupillary  symptoms  which  have  been  united  under  the 
name  of  Horner's  symptom-complex.  The  relative  frequency  of  this  syndrome 
depends  upon  the  fact  that  gliosis  and  syringomyelia  have  a  strong  predilection 
for  the  level  of  the  lower  cervical  cord.  At  the  level  of  the  8th  cervical  seg- 
ment in  the  gray  substance,  however,  is  located  the  so-called  "cilio-spinal  center" 
from  which  fibers  proceed  by  way  of  the  anterior  roots  of  the  8th  cervical  and 
two  upper  dorsal  nerves  into  the  lower  ganglion  of  the  cervical  sympathetic, 
from  which  by  another  set  of  neurones  there  is  further  conduction  to  the  superior 


SPINAL    GLIOSIS    AND    SYRINGOMYELIA  147 

tarsal,  the  orbital  and  the  dilator  pupillae  muscles.  The  first  mentioned  is  made 
up  of  the  involuntary  muscular  fibers  of  the  levator  palpebrae;  the  second  bridges 
over  the  lower  portion  of  the  orbital  fissure  and  so  prevents  the  contents  of  the 
oibit  from  sinking  downward  and  backward;  the  third  is  the  antagonist  of  the 
sphincter  pupillae,  innervated  from  the  motor  oculi.  The  cutting  off  of  influ- 
ences from  the  cilio-spinal  center  is  manifested,  1,  by  a  paralytic  mvosis  in 
which  the  pupil  narrowed  on  account  of  paralysis  of  the  dilator  fibers,  does  not 
enlarge  when  the  eye  is  shaded;  2,  by  a  narrowing  of  the  palpebral  fissure,  the 
so-called  "sympathetic  ptosis";  3,  by  a  sinking  of  the  eyeball  back  into  the 
orbit,  the  so-called  "enophthalmus.''  This  last  component  of  Horner's  symp- 
tom-complex is,  indeed,  not  always  definitely  to  be  found. 

Obviously  this  symptom-complex  can  also  occur  in  lesions  of  the  cervical 
sympathetic,  of  the  anterior  roots  of  the  lowest  cervical  and  two  upper  thoracic 
nerves  and  their  rami  comniunieantes  ;  we  have  met  it  as  an  accompaniment  of 
the  "lower  armplexus  paralysis"  ("Klumpke's  paralysis")  already  in  Lecture 
I.     (See  also  Lecture  XXV.) 

Further,  spastic  symptoms  which  owe  their  origin  to  the  involvement  of 
the  lateral  columns  by  the  pathological  process,  are  not  at  all  infrequent,  par- 
ticularly in  the  lower  extremities,  as  in  the  arms,  atrophy  goes  hand  in  hand 
with  loss  of  reflexes;  exaggeration  of  the  patellar  and  Achilles  reflexes  are  found  ; 
often,  also,  Babinski's  phenomenon  and  ankle  clonus;  the  hypertonia  of  the 
muscles  only  seldom  reaches  such  a  degree  that  the  characteristic  gait  of  spastic 
spinal  paralysis  occurs. 

Both  the  oculo-pupillary  and  the  spastic  phenomena  usually  are  limited  to 
one  side. 

Of  the  atypical  forms  of  syringomyelia  we  will  give  a  short  clinical  outline 
of  "syringobulbia"  already  mentioned;  in  this,  there  are  found  (often  only  on 
une  side)  dissociated  sensory  paralysis  in  the  distribution  of  the  trigeminus, 
atrophy  of  the  tongue  or  masticatory  muscles,  recurrens  paralysis,  formation 
of  ulcers  on  the  posterior  wall  of  the  pharynx,  paralysis  of  the  eye  muscl  s, 
ageusia,  etc. 

Differential  Diagnosis 

The-  differentiation  of  syringomyelia  from  progressive  spinal  muscular 
atrophy,  since  as  far  as  the  muscle  symptoms  go,  they  can  greatly  resemble 
one  another,  depends  upon  the  demonstration  of  the  typical  dissociation  of 
sensation.  Also  the  trophic  disturbances  found  eventually  in  the  AranrDuchenne 
disi  .use  are  of  much  les>  intensity  than  those  df  syringomyelia.  In  spastic 
s.  i  mgomvehi  the  determination--^!  I  lie  condition  of  sensibility  likewise  furnishes 
tin-  means  of  deciding  between  it  and  amyotrophic  lateral  sclerosis.  The  differ- 
ential diagnosis  between  syringomyelia  and  lepra  anesthetics  can  sunn  limes  be 
m tv  difficult,  a  question  which  in  our  neighborhood,  fortunately,  scarcely  ever 
conns  up.  I  It  does  in  the  United  States.  Translator.]  Decisive  as  In  lepra 
is  the  recognition  of  nodular  thickenings  of  the  peripheral  nerve  trunks,  par 
ticularly,  however,  the  finding  of  the  Armauer  Hansen  bacillus. 

Trophic  disturbances  aboul  the  face  occur  frequently  in  lepra,  very  rarely 
in  syringomyelia;  for  lepra  speaks  further  the  finding  of  skin  areas  with  in- 


148  LECTURE    X 

creased  or  decreased  pigment,  in  which  sensibility  is  diminished  (insular  anes- 
thesia or  hypesthesia)  as  well  as  the  occasional  occurrence  of  febrile  paroxysms. 
A  rare  disease  isolated  by  Charcot  and  Joffroy,  hypertrophic  cervical 
pachymeningitis  can  produce  clinical  pictures  very  similar  to  syringomyelia. 
Pathologico-anatomically  it  consists  in  a  great  proliferation  of  the  meninges 
in  the  cervical  region  which  finally  surround  the  cord  like  a  thick  cicatricial  band 
which  cannot  be  separated  from  the  cord  proper.  Contractions  within  this  great 
connective  tissue  envelope  lead  to  solutions  of  continuity  within  the  gray  matter 
of  the  spinal  cord,  which  evidence  themselves  in  dissociated  anesthesia,  and  to 
lesions  of  the  pyramids  recognizable  by  spastic-paretic  phenomena.  Almost 
regularly  the  "preacher's  hand"  already  mentioned,  which  may  depend  partly 
upon  secondary  alterations  in  the  anterior  horn  and  partly  upon  changes  in 
the  motor  roots,  is  found.  The  regular  involvement  of  the  sensory  roots  in  the 
meningeal  thickening  leads  without  exception  to  a  neuralgiform  initial  stage  of 
the  disease,  upon  which  in  its  differential  diagnosis  from  syringomyelia,  most 
importance  is  to  be  placed.  In  the  late  stages  of  the  disease  paralysis  of 
sphincters,  formation  of  bed-sores  and  marked  spastic  paraplegia  of  the  legs 
appear  and  the  picture  loses  its  resemblance  to  syringomyelia,  to  pass  over  more 
and  more  into  that  of  compression  of  the  spinal  cord. 

Course  and  Prognosis 

As  a  rule,  the  first  beginning  of  the  disease  is,  as  already  said,  so  little 
marked,  that  the  patients  011I3'  late — that  is,  when  they  become  frightened  by  the 
occurrence  of  muscular  atrophy  or  trophic  skin  and  bone  disturbances — come 
to  a  physician,  usually  between  the  ages  of  20  and  25  years.  Nevertheless,  the 
presence  of  the  disease  in  childhood  can  be  affirmed,  not  only  retrospectively 
from  a  history  of  analgesia,  etc.,  but  is  sometimes  directly  determined  by  a 
physician,  the  last  indeed  seldom  enough.  The  further  development  of  the  dis- 
ease proceeds  extremely  slowly ;  exacerbations  of  rapid  progress  and  long  sta- 
tionary periods  alternate  with  one  another.  As  to  life,  the  prognosis  is  in 
general  good ;  the  patients  can  live  to  be  old,  in  any  case  they  only  rarely 
succumb  to  the  syringomyelic  paralysis  of  the  muscles  of  deglutition  or  of 
those  of  respiration,  sepsis,  etc.,  but  die  usually  of  some  intercurrent  disease. 
As  to  recovery,  the  outlook  is  naturally  poor;  nevertheless,  trophic  lesions  can 
heal  up  not  to  return  again. 

Treatment 

In  the  treatment  of  syringomyelics,  prophylaxis  on  the  one  hand  against 
overexertion,  injurious  climatic  effects,  etc.,  on  the  other  against  wounds  and 
burns,  plays  the  chief  role.  Against  the  disease  itself  occasional  arsenic  cures 
or  galvanization  of  the  spinal  cord  can  be  tried.  For  the  technique  of  this  last, 
see  Lecture  VII,  page  116.  The  results  which  have  been  described  from  the 
subjection  of  the  cervical  cord  to  the  X-ray  are  not  very  convincing,  on  account 
of  the  well-known  tendency  of  the  disease  to  come  spontaneously  to  a  standstill; 
nevertheless,  further  experiences  must  be  awaited  before  forming  a  definite 
opinion  as  to  this  new  method. 


HEMATOMYELIA  119 


B.    Hematomyelia 


Hematomyelia,  or  hemorrhage  into  the  spinal  cord  occurs  rarely  spon- 
taneously, relatively  frequently  as  the  result  of  trauma.  In  the  first  case  it 
is  either  the  result  of  rupture  of  a  vessel  on  account  of  pathological  brittleness 
of  the  arterial  wall  (arteriosclerosis,  syphilis,  pernicious  anemia)  in  which 
usually  we  find  given  as  causes  factors  which  raise  the  blood  pressure,  like  sneez- 
ing, straining,  coitus,  etc.,  or  the  hemorrhage  follows  secondarily  in  an  already 
diseased  tissue. 

I  saw  one  case  (published  by  Gerhardt)  in  which  the  hemorrhage  occurred 
in  an  intramedullary  glioma,  which  up  to  this  time  had  remained  entirely  latent. 
Also  in  acute  and  chronic  myelitis  secondary  spontaneous  hemorrhages  have 
been  described. 

Traumatic  hematomyelia  occurs  in  severe  contusions  of  the  spinal  column, 
injuries  by  lightning  or  electrocution  by  powerful  currents;  also,  as  a  result  of 
certain  operative  measures  in  pathological  labors  (difficult  forceps  extractions, 
the  Veit-Smellie  manipulation),  or  in  Scliultzc's  method  of  artificial  respiration 
in  infants  born  asphyxiated.  Further,  by  forced  bending  forward  or  backward 
of  the  cervical  spine  (prize-fighters,  ''jiu-jitsu,"  etc.). 

In  typical  cases  the  patient  experiences  a  sudden  and  severe  pain  in  the 
region  of  the  vertebral  column  corresponding  to  the  hemorrhagic  focus  and  falls 
to  the  ground  with  more  or  less  marked  paralysis  of  the  muscles  distal  to  this 
point.  Less  frequently  there  is  a  somewhat  slower  development  of  the  motor 
symptoms  to  which  also  disturbance  of  sensibility  and  of  the  bladder  and  rectum 
are  usually  added.  A  great  part  of  these  symptoms  do  not  depend  upon  the 
direct  destruction  of  the  elements  of  the  spinal  cord  by  the  extravasated  blood, 
but  usually  represent  remote  effects  of  the  hemorrhage  which  are  alluded  to 
as  "shock,"  "inhibition,"  "'diaschisis  phenomena."  These  are  in  their  nature 
temporary,  so  usually  in  the  days,  sometimes  in  the  hours,  next  succeeding  the 
hemorrhage,  partial  subsidence  of  the  symptoms  occurs.  Since  now  the  hemor- 
rhage is  usually  in  the  central  region  the  predominating  syndrome  of  hema- 
tomyelia often  presents  the  greatest  analogies  to  the  syringomyelic  symptom- 
complex,  particularly  in  the  specially  frequent  cases  in  which  the  location  of 
the  hemorrhage  is  in  the  cervical  region;  then  .along  with  spastic  phenomena  in 
the  lower  limbs,  atrophy  of  the  small  muscles  of  the  hand,  with  the  "ape-hand" 
or  "claw-hand"  deformity,  a  typical  dissociated  anesthesia  and  the  Horner 
oculo-pupillary  symptom-complex  can  lie  found  (see  page  1  Hi).  Making  of  a 
prognosis  is  naturally  only  possible  after  the  remote  symptoms  have  cleared 
up.  It  depends  mainly  upon  the  location  of  the  lesion;  if  the  hemorrhage  has 
occurred  high  up  in  the  cervical  region  the  prognosis  is  very  gloomy,  since  in 
consequence  of  its  nearness  to  the  medulla,  paralysis  of  the  heart  and  respira- 
tion with  high  fever  is  apt  to  occur.  Also  location  low  in  the  spinal  cord  has 
\'r\  evil  results,  since  permanent  paralysis  of  the  bladder  carries  with  it  the 
Sanger  of  ascending  infection  of  tit*-  urinary  apparatus.  Many  cases  of  hema- 
tomyelia high  up  in  the  cord  perish  rapidly  in  shock.  On  the  other  hand,  small 
hemorrhages  in  the  dorsal  and  lumbar  regions  can  recover  almost  without 
residual   symptoms. 


150 


LECTURE    X 


Therapeutically,  in  the  early  stages  absolute  rest  is  the  chief  thing.  Care 
must  be  taken  that  through  the  use  of  purgatives  movement  of  the  bowels  takes 
place  without  straining,  that  is  without  any  considerable  elevation  of  the  blood 
pressure.  Bleeding,  or  a  series  of  leeches  or  wet  cups  along  the  spine  can  be  of 
use  under  some  circumstances.     The  patients  must  not  get  up  before  the  end  of 

four  weeks !  The  residual  parescs, 
sphincter  disturbances,  etc.,  often  need 
symptomatic  treatment;  we  need  not  go 
into  these  things  here,  since  they  are 
considered  elsewhere. 

C.    Transverse  Lesions  of 
the  Spinal  Cord 

Considered  from  a  clinical  point  of 
view,  discussion  of  a  rather  hetero- 
geneous array  of  disease  conditions 
under  this  unique  designation,  is  en- 
tirely justified;  since  disease  or  injury 
of  the  cross-section  of  the  spinal  cord, 
whether  it  owes  its  origin  to  a  myelitis, 
to  a  traumatic  solution  of  continuity,  to 
compression  by  extra-medullary  tumors 
or  tuberculous  spondylitis,  or  to  the 
development  of  a  neoplasm  in  the  spinal 
cord  itself,  produces  symptom-com- 
plexes agreeing  to  the   fullest   extent.* 

On  this  account  I  intend  deviating 

from  the  usual  method,  first  to  present 

to  you  the  facts  common  to  the  group 

of  disease  conditions  indicated,  then,  in 

order  to  avoid  repetition,  I  will  expose 

the    individual    pathogenetic    sub-varie- 

pIG  48  ties     according     to     their     particular 

Extent  of  the  Anesthesia  in  Transverse       pathologico-anatomical  and  clinical  as- 

Lesions  at  Different  Levels.  pects. 


1.     General  Symptomatology  of  "Transverse  Lesions" 

A  lesion  of  the  spinal  cord  which  so  severely  injures  the  total  cross  section 
of  this  cylindrical  organ  at  a  definite  level,  that  the  conduction  of  impulses  by 

*  The  same  remarks  apply  to  the  so-called  "concussion  of  the  spinal  cord,"  which  though 
anatomically  still  little  investigated,  can  give  clinically  very  suggestive  pictures.  -I.  Visscher 
has  communicated  to  me  from  the  Balkan  War  some  interesting  observations  of  typical  "trans- 
verse lesions"  which  had  occurred  without  any  direct  traumatism  in  soldiers  in  whose  neigh- 
borhood a  grenade  had  burst  or  even  had  passed  close  by  them. 


TRANSVERSE    LESIONS    OF    THE    SPINAL    CORD 


151 


all  the  fiber  tracts  is  here  interrupted,  causes,  of  course,  entire  loss  of  sensi- 
bility and  motility  in  the  portions  of  the  body  distal  to  this. 

Fig.  48  shows  the  extent  of  anesthesia  in  total  and  symmetrica]  transverse 
lesions  at  different  levels.  The  extent  of  motor  paralysis  is  shown  in  the  fol- 
lowing tables  after  E.  ViUiger.  For  every  assumed  transverse  lesion,  the  com- 
plex of  muscles  paralyzed  is  here  exposed  (those  in  the  column  representing  the 
affected  segment  and  all  those  to  the  right  of  this  column).  In  injuries  of  the 
spinal  cord  (dislocation  or  fracture  of  the  vertebral  column)  besides  this  paral- 
ysis, irritative  symptoms  from  the  region  immediately  above  the  lesion  come  into 
consideration  in  the  clinical  estimation  of  the  case.  So,  for  example,  as  shown  in 
Fig.  49  in  destruction  of  the  7th  cervical  segment,  as  a  rule,  the  forearms  are 


Complete  Severing  of  the  Spinal  Cord  at  the  Level  of  the  Vllth  Cervical  Segment. 
(Vertebral  Fracture.) 


held  in  forced  flexion  by-  contracture  of  the  biceps  and  the  brachialis  anticus 
muscles. 

In  total  solutions  of  continuity,  anesthesia  extends,  of  course,  to  all  quali- 
ties of  sensation;  indeed,  all  centripetal  conduction  is  suspended;  the  motor 
paralysis,  too,  is  absolute  (no  paresis,  but  paralysis),  since  there  is  severing 
not  only  of  the  chief  motor  tracts,  that  is  the  pyramids,  but  of  each  and  everj 
Connection  between  the  organ  of  the  will  and  the  spinal  centers  of  movement. 
Now  as  to  the  character  of  the  paralysis:  according  to  what  we  have  said  in 
Lecture  VII  (page  112),  a  spastic  paralysis  would  be  expected,  as  below  tin- 
level  of  the  separation  the  reflex  arcs  are  intact  and  freed  by  the  lesion  from 
inhibiting  cortico-spinal  influences.  In  complete  transverse  separation  of  con- 
tinuity in  the  upper  parts  of  the  spinal  cord,  however,  an  absolute  atonia  and 
arcfli  \ia  in  the  paralyzed  region  is  found  almost  without  exception,  at  any 
rale,  in  the  early  stages.  It  has  been  attempted  to  explain  this  as  due  to  shock  ; 
tin-,  however,  is  not  satisfactory  since,  first,  in  many  cases  atonia  and  areflexia 


152 


LECTURE    X 


s 

3 

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eg 

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es 

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to 
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ill 

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q 

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3 

a 

3 

Of 

CO 
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a 

a 
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a 

a 

la 

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C 

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-5- 

CO 

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3 

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V 

OS 

X 

c~ 

0 

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72 

CO 

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a 

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r~ 

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CO 

0 

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>3 

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P 

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C>] 

Short 

deep 

neck 

muscles. 

- 

TRANSVERSE    LESIONS    OF    THE    SPINAL    CORD 


153 


Segmental  innervation  of  the  arm  muscles. 



Cervical  segments. 

Thorac 

ic  segm. 

5                          6                          7                          8 

1 

S 
o 
CO 

Supraspinal 

Teres  min. 

1 

Deltoideus 

Infraspinatus 

Subscapularis 

1 

Teres  majoi 

a 

< 

Biceps 

Brachialis 

Coracobraehialis 

1 

Triceps  brach. 

Anconaeus 

a 

« 
u 

o 

Supinator  long. 

Supinator  brevis 

Extensor  carpi  radial. 

Pronator  teres 

Flexor  carpi  radial. 

Flexor  pollic.  long. 

Abduct,  poll.  long. 

Extens.  poll.  brev. 

Extens.  poll.  long. 

Extens.  digit,  coram. 

Extens.  indie,  prop. 

Extens.  carpi  uln. 

Extens.  digit.  V  prop. 

Flex,  digitor.  sublimis. 

Flex,  digitor.  profund. 

Pronator  quadrat. 

Flexor  carpi  uln. 

Palniaris  long. 

•6 

c 

a 

X 

Abduct,  poll.  brev. 

Flex.  poll.  brev. 

Opponens  poll. 

Flexor  digit.  V 

(Ipimnens  dig.V 

Adduct.  poll. 

Palmaris  brev. 

Abductor  dig.-? 

Lumbricales 

[nteroBsei 

154 


LECTURE    X 


Segmental  innervation  of  the  leg  muscles. 

Th„      |        L,        |        L,               L3                Lt        |        L5                S,               S2 

a, 

X 

H 
to 

1 
fa 

Ileopsoas 

Tensor  fasciae       | 

Glutaeus  medius 

Glutaeus   minim. 

Quadratus  femoris 

Gemellus  inferior 

Gemellus  super. 

Glutaeus  maxim. 

Obturator  intern. 

Pvriioriuis 

Sartorias 

Pectineus 

Adduct.  Ions. 

Quadriceps 

Gracilis 

Adductor  brevis 

Obturator  ext. 

! 

Adduct.  magn. 

Adduct.  minim. 

Articularis  gen. 

Semitendinosus 

•Semimembranosus 

Biceps  femoris 

Tibialis  ant.    | 

Extensor  halluc.  long.     | 

Popliteus 

Plantaris 

Extensor  digit,  long. 

Soleus 

Gastrocnemius 

Peroneus  longus 

Peroneus  brevis 

Tibialis  postic. 

Flexor  dig.  long. 

Flexor  halluc.  long. 

Extensor  halluc.  brev.     | 

Extensor  digit  brevis. 

Flex.  dig.  brev. 

Abduct,  hall. 

Flex,  halluc.  brev. 

Lumbricales 

Abduct   hall. 

Abduct,  dig.  V 

Flexor  dig.  V  br. 

( Ipponens  dig.  V 

Quadrat,  plant. 

Interossei 

TRANSVERSE    LESIONS    OF    THE    SPINAL    CORD 


155 


remain  permanently;  second,  they  may  occur  also  in  non-traumatic  cases  (for 
example,  in  transverse  myelitis).  Probably  it  is  the  result  of  the  disturbances 
of  lymph  and  blood  circulation  occurring  in  complete  transverse  affections  high 
up  in  the  cord  which  lead  to  serious  functional  injury  of  the  posterior  roots 
and  anterior  horn  cells  in  the  lower  levels. 

Complete  transverse  lesions  of  the  spinal  cord  always  lead  to  bladder  and 
rectal  disturbances;  there  is  retention  of  stools;  in  the  bladder,  retention  of 
urine  is  usually  the  most  prominent  symptom;  when,  however,  the  distention  of 
the  bladder  has  reached  a  certain  degree,  there  can  be  a  reflex  involuntary  dis- 


FlO,  50. 

The  Brown  Signard  Symptom-Complex. 


charge  of  urine  ("intermittent  incontinence").  Only  in  lesions  which  are  sit- 
uated very  low  down  and  destroy  the  vesico-spinal  and  ano-spinal  centers  of 
the  gacral  region,  other  symptoms  occur;  either  a  continued  dribbling  of  the 
urine  ("permanent  incontinence")  or  discharge  of  the  urine  in  drops  from  the 
moment  the  filling  of  the  bladder  has  reached  a  certain  degree  ("'ischuria 
paradoxa").  Further,  incontinence  of  the  bowels.  Of  vasomotor  and  trophic 
disturbances  are  to  be  mentioned  the  dilatation  of  vessels  to  be  observed  in 
the  paralyzed  region  in  fresh  cases,  which  later  gives  wav  to  conditions  of  cold- 


156 


LECTURE    X 


ness   and   cyanosis   and  the  marked  tendency   to   deep   and  rapidly   spreading 
bed-sores. 

Of  great  prognostic  importance  is  the  differentiation  between  partial  and 
total  transverse  lesions.  In  making  this,  the 
following  points  are  to  be  considered:  In  in- 
complete transverse  lesions  the  paralysis  is  not 
symmetrical  as  in  the  complete  ones  and  has, 
further,  the  tendency  to  partial  recovery 
(naturally,  provided  the  cause  ceases)  ;  also 
when  the  lesion  is  located  high,  the  patellar 
reflexes  are  never  permanently  lost,  but,  as  a 
rule,  exaggerated.  Frequently  there  is  a  differ- 
ence between  them  on  the  right  and  on  the  left. 
Vasomotor  and  sphincter  disturbances  are  only 
present  to  a  slight  degree.  Further,  irritative 
symptoms  may  be  evident  distal  from  the  lesion 
(pains,  twitching  of  the  muscles  in  injuries  to 
the  cord,  also  permanent  erection  of  the  penis, 
the  so-called  "priapism"). 

The  phenomena  which  occur  in  an  only  half- 
sided  transverse  lesion  of  the  spinal  cord,  most 
usually  from  trauma  or  in  tumor  formation, 
make  up  what  has  been  called  the  "Broun- 
Sequard  symptom  complex."  This  is  composed 
of  the  following  symptoms  which  present  them- 
selves in  the  parts  of  the  body  distal  to  the 
seat  of  the  lesion :  On  the  side  of  the  lesion  motor 
paralysis,  disturbance  of  the  deep  sensibility 
and  hyperesthesia  for  touch ;  on  the  opposite 
side  disturbance  of  the  superficial  sensibility, 
namely  for  pain  and  temperature  stimuli. 

The  motor  paralysis  on  the  side  of  the 
lesion  has  naturally  a  spastic  character ;  its 
method  of  production  is  readily  understandable 
from  Fig.  50.  The  disturbance  of  the  deep 
sensibility  (marked  involvement  of  the  sense  of 
position,  ataxia)  on  the  same  side,  also  con- 
sidered in  that  diagram,  is  explained  by  the 
almost  exclusively  homolateral  ascent  of  the 
paths  for  "bathvesthesia,"  that  is,  of  the  pos- 
terior columns  and  the  direct  cerebellar  tracts. 
Also  vibration  sense  is,  as  I  have  been  able  to 
recognize,  disturbed  or  lost  on  the  side  of  the 
lesion  in  Broum-Sequard's  paralysis.  The  crossed  superficial  anesthesia  affects 
always  pain  and  temperature  sense,  since  conduction  of  these  qualities  of  sensa- 
tion is  almost  exclusively  carried  out  through  fibers  passing  over  to  the  other 
half  of  the  spinal  cord.     In  typical  cases  touch  sense  is  altered,  too,  but  much 


Broirn-Stqiicird's  Symptom-Complex. 
Lesion  of  the  Left  Half  of  the 
Spinal   Cord. 

Disturbances  of  Sensibility. 
MB  ro  ithesia. 

=  Deep  Anesthesia. 

=  Superficial  Hyperesthesia. 


TRANSVERSE    LESIONS    OF    THE    SPINAL    CORD  157 

less  intensely.  This,  also,  is  explainable  from  the  anatomico-physiological  con- 
ditions. Tactile  impressions  are  conducted  toward  the  brain  partly  uncrossed 
(in  the  posterior  columns)  partly  crossed  (in  the  lateral  columns).  Sometimes 
there  is  neither  tactile  anesthesia  nor  hypoesthesia.  The  explanation  of  super- 
ficial hyperesthesia  on  the  same  side  (which  is  only  a  temporary  symptom)  is 
difficult.  Probably  after  a  one-sided  lesion,  the  cells  of  the  posterior  horn, 
which  are  intercalated  in  the  crossed  sensory  tracts  are  loaded  down  with  too 
much  work  (since  now  only  these  crossed  tracts  are  open  to  tactile  stimuli, 
part  of  which  normally  pass  up  in  the  uncrossed  tracts)  and  this  overwork  mani- 
fests itself,  until  the  organ  has  become  accustomed  to  the  new  relations,  by 
touch  stimuli  being  perceived  as  painful. 

If  the  unilateral  lesion  is  located  in  the  cervical  region  we  have  a  "spinal 
hemiplegia,"  if  it  is  in  the  dorsal  region,  a  "hemiparaplegia."  Fig.  51  illustrates 
such  a  case.  The  narrow  anesthetic  zone  above  the  region  of  motor  paralysis 
must  be  attributed  to  destruction  of  the  posterior  roots  entering  the  affected 
region  of  the  cord.  Typical  "classic"  forms  of  the  Brown-Sequard  symptom- 
complex  are,  however,  much  rarer  than  atypical  ones  in  which  only  an  incom- 
plete half-sided  lesion  or,  on  the  contrary,  one  also  affecting  the  other  half  of 
the  spinal  cord,  is  present. 


2.     DIFFUSE    MYELITIS 

We  begin  our  consideration  of  the  individual  affections  of  the  spinal  cord 
leading  to  transverse  lesions  with  Diffuse  Myelitis,  that  is,  with  that  inflamma- 
tory process,  which  in  its  diffusion  in  the  diseased  organ  is  not  limited  to  definite 
anatomical  elements  (in  contradistinction,  for  example,  to  "poliomyelitis  an- 
terior," which,  as  we  will  see,  does  not,  as  a  rule,  pass  the  boundaries  of  the 
anferior  horns,  and  which  also  does  not  occur  in  diffused  foci  (like  disseminated 
myelitis  already  mentioned  in  Lecture  IX). 

The  myelitides  present  the  following  pathologico-anatomical  characters: 
In  very  fresh  cases,  macroscopically  the  spinal  cord  in  the  affected  regions  ap- 
pears swollen  and  somewhat  red  in  color;  in  the  advanced  disease  it  may  be 
softened  to  a  discolored  pulp.  Microscopically  the  early  stage  of  the  disease 
is  characterized  by  cloudy  swelling  and  vacuolization  of  the  ganglion  cells,  with 
loss  of  their  finer  structure  as  well  as  of  the  nerve  fibers  arising  from  them; 
further,  by  great  dilatation  of  the  vessels,  minute  extravasations  of  blood,  in- 
filtration of  round  cells,  crowding  of  the  perivascular  lymph  sheaths  with  granu- 
lar cells,  etc.  In  the  later  stages  of  the  disease  the  infiltrative  processes  pro- 
gressively decrease  and  "reparatory"  proliferative  processes  in  the  neuroglia 
occur.  Above  and  below  the  inflammatory  foci  the  so-called  "secondary  de- 
generations" take  place  in  thcdong  fiber  tracts  of  the  spinal  cord  ;  following 
"Waller's  law"  (see  page  10),  the  degeneration  in  the  pyramidal  tracts  is  de- 
scending, that  in  the  posterior  columns  and  in  the  spino-cerebellar  tract,  ascend- 
ing (see  Fig.  5"2).  Purulent  myelitis,  in  which  there  is  breaking  down  of  the 
spinal  cord  with  pus  formation  and  sometimes  an  abscess  membrane,  is  exces 
sively  rare.    Very  infrequent  also  are  the  subchronic  and  chronic  diffuse  myeli- 


158 


LECTURE    X 


tides  in  which  parenchymatous  destruction  is  combined  from  the  start  with  pro- 
liferative alterations  of  the  neuroglia  and  of  the  vessel  walls. 

From  an  etiological  point  of  view  the  diffuse  myelitides  are  quite  hetero- 
genous. The  acute  forms  can,  as  a  whole,  be  considered  infectious ;  usually 
they  are  connected  with  general  diseases  (influenza,  typhoid  fever,  acute  rheu- 
matism, variola,  erysipelas,  malaria,  pneumonia,  diphtheria,  dysentery,  gono- 
coccemia,  measles,  syphilis).  In  the  so-called  "primary  or  idiopathic  acute 
myelitis,"  for  which  sometimes  exposure  to  cold,  getting  wet,  and  overexertion 
have  been  held  responsible,  most  probably  some  obscure  infection  is  always  re- 


Tracts  which  Degenerate  Upward. 
I.   Long  fibers  from  the  posterior  roots. 
II.  Tractus     spinocerebellars      (a,     poster 
direct     cerebellar     tract;     b,     anter 
Gowcrs1   column). 
III.  Tractus   spino-tha! 


Tracts   which  Degenerate   Downward. 

1.  Tractus  cortico-spinalis,  pyramidal  tract,  chief 
motor  path  (a,  lateral  pyramidal  tract; 
b,   anterior   pyramidal    tract). 

2-5.  Further  motor  tracts,  subcortico-spinal  tracts 
i -.  tractus  rubrospinalis  and  thalanm 
spinalis;  3,  tractus  vestibulo-spinalis;  4. 
tractus   tectospinalis). 

5.    Descending  fibers  of  the  posterior  column. 


dorsal 


sponsible,  and  the  factors  mentioned  have  only  acted  as  exciting  causes.  In 
fact,  streptococci,  staphylococci,  and  colon  bacilli  have  occasionally  been  recog- 
nized in  the  diseased  spinal  cord.  On  the  other  hand,  it  seems  certain  that 
infectious  myelitides  can  occur  also  without  microbic  invasion  of  the  organ, 
usually  from  the  action  of  toxines.  For  the  subacute  and  chronic  forms, 
syphilis  comes  particularly  into  question  as  a  causal  factor;  occasionally,  also, 
exogenic,  chronic  intoxications,  as  saturnism  and  alcoholism.  Abscess  of  the 
spinal  cord  occurs  almost  only  from  metastasis,  particularly  after  ulcerative 
endocarditis.  A  special  position  belongs  to  those  forms  of  myelitis  which  occur 
in  connection  with  multiple  gas  embolisms  in  the  vessels  of  the  spinal  cord ; 
these  have  been  observed   in  consequence  of  the  development  of  bubbles  of  air 


TRANSVERSE    LESIONS    OF    THE    SPINAL    CORD  159 

in  the  blood  upon  abruptly  passing  from  a  high  to  a  low  atmospheric  pressure. 
Workers  on  bridge  building  and  >n  tunnels  are  particularly  exposed  to  this 
"caisson  paralysis"  or  "diver's  paralysis"  if  special  arrangements  for  a  gradual 
"decompression"  have  not  been  provided.  The  development  and  course  of 
transverse  myelitides  may  be  summed  up  as  follows:  usually  a  prodromal  stage 
which  is  characterized  by  pain  in  the  back,  paresthesias  in  girdle  form,  sensa- 
tion of  drawing  and  formication  in  the  extremities  along  with  general  malaise, 
shivering  and  rise  of  temperature,  precedes  the  development  of  paraplegia. 
Only  very  rarely  is  the  beginning  abrupt,  "apoplectiform."  A  very  slow  be- 
ginning is  not  frequent  and  is  to  be  considered  prognostically  unfavorable  as 
to  restoration  of  function  (provided  that,  syphilis,  against  which  we  can  act 
energetically,  is  not  at  fault).  On  the  other  hand  the  acute  forms  sometimes 
recover  in  astounding  fashion.  The  longer  the  fever  lasts  the  more  unfavorable 
is  the  prognosis;  it  is  further  mainly  dependent  upon  the  seat  of  the  transverse 
lesion.  Myelitides  of  the  cervical  region,  on  account  of  their  proximity  to  the 
medulla  and  its  centers  so  important  to  life,  is  always  very  dangerous.  The 
occurrence  of  broncho-pneumonia,  large  bed-sores,  pyelo-nephritis,  and  the 
tendency  of  the  disease  process  to  extend  further  (ascending  paralysis)  are  to 
be  considered  as  prognostically  unfavorable  criteria.  Interesting  in  a  case 
of  lumbo-sacral  myelitis  observed  by  me  was  the  circumstance  that  the  patient 
afterward,  in  spite  of  complete  motor  and  sensory  paraplegia,  went  through 
two  labors,  normal  except  for  complete  painlessness. 

According  to  the  symptomatology  we  can  distinguish  a  number  of  varieties. 
First,  according  to  the  level  of  the  spinal  cord  in  which  the  transverse  lesion  has 
occurred,  a  lumbo-sacral,  dorsal  and  cervical  form,  individual  forms  which  in 
clinical  importance  are  inferior  to  ascending  myelitis,  in  which  after  beginning 
in  the  lumbo-sacral  region  continually  higher  levels  are  involved  in  the  motor 
and  sensory  paralysis.  In  this,  the  inflammation  extends  by  way  of  the  peri- 
vascular lymph-sheaths  upward,  as  numerous  animal  experiments  conducted  by 
V.  Salic,  under  my  direction,  have  shown.  The  central  canal  plays  a  very 
subordinate  role.  Cases  in  which  the  ascent  occurs  in  exceedingly  violent  and 
Steady  manner,  so  that  within  a  few  days  from  their  commencement  deatli  fol- 
lows from  paralysis  of  the  muscles  of  deglutition  and  of  respiration,  are  classed 
with  the  so-called  ''La/ndry's  paralysis,"  or  "paralysis  ascendens  acutissima." 
They  belong  properly,  however,  with  affections  which  pathogenetieally  must  be 
Separated  from  transverse  myelitis,  namely  with  the  ascending  cases  of  polio- 
myelitis anterior  which  come  under  the  head  of  the  Hcinc-McJiii  disease  (  see 
Lecture  XVI)  and  particularly,  also,  with  those  of  polyneuritis  acutissima.  As 
"myelitis  migrans"  I  have  described  a  peculiar  case  in  which  a  lumbo-sacral 
transverse  paralysis,  which  had  arisen  acutely  and  was  to  he  attributed  to  chill- 
ing and  infection,  recovered.  Immediately  afterward,  however,  paretic  symp- 
toms and  disturbances  of  sensibility  occurred  in  the  trunk.  These  symptoms 
now  ascended,  in  a  step-like  manner  to  a  certain  extent,  upward,  with  recrudes- 
cence of  the  fever,  while  at  the  same  time  from  below,  a  return  to  the  normal 
Occurred,  likewise  by  steps.  After  the  arms  had  been  affected  and  already  had 
begun  to  get  better,  the  situation  became  extraordinarily  dangerous  on  account 
of  the  occurrence  of  par   stliesia  in  the  region  of  the  trigeminus  and  violent  hie- 


160  LECTURE    X 

cough.  At  this  critical  moment  the  process  came  finally  to  a  stop,  and  (in 
spite  of  the  duration  of  the  progressive  period  for  weeks)  at  length  recovered. 
This  conduct  can  well  be  compared  with  that  of  '"migrating  erysipelas." 

From  a  therapeutic  point  of  view,  active  attack  upon  the  cause  is  only 
possible  in  those  forms  depending  upon  malaria  and  syphilis.  In  a  typical  case 
of  very  severe  syphilitic  lumbo-sacral  myelitis  I  saw  a  most  startling  effect 
from  an  intramuscular  injection  of  0.6  grin,  of  salvarsan.  Six  days  after  the 
injection  the  patient  began  to  move  the  previously  totally  paralyzed  legs  again; 
on  the  thirteenth  day  he  could  stand,  and  in  the  end  (after  several,  this  time 
intravenous,  injections  of  salvarsan)  there  was  complete  recovery.  It  may  be 
attempted  to  treat  the  other  infections  by  injections  of  colloidal  silver  (col- 
largol,  elektrargol).  Whether  these  really  are  of  value,  or  if  in  the  cases  which 
have  recovered  under  this  treatment  it  was  merely  an  accidental  coincidence,  can- 
not yet  be  decided.  For  the  rest,  we  must  content  ourselves  with  symptomatic 
treatment  and  care.  Prevention  of  bed-sores  by  frequent  change  of  position, 
protecting  dressings,  bathing  with  alcohol,  with  subsequent  application  of 
ointment  of  balsam  of  Peru,  rubber  rings,  water-cushions,  etc.,  prevention  of 
cystitis  by  the  most  careful  asepsis  on  catheterizing  (which  is  only  to  be  done 
when  it  cannot  be  avoided),  giving  urotropin,  etc.  Complications  which  have 
already  arisen  (bed-sores,  cystitis,  broncho-pneumonia)  are  to  be  treated  with 
all  care.  To  the  treatment  of  the  paralyses,  what  has  already  been  said  in 
the  discussion  of  spastic  spinal  paralysis,  syringomyelia,  etc..  applies ;  I  would 
refer  you  also  to  my  later  remarks  in  Lectures  XVI  and  XXII. 

3.     TUMORS    OF    THE    SPINAL    CORD 

Under  tumors  of  the  spinal  cord,  in  a  clinical  sense,  we  include  both  new 
formations  developing  in  the  spinal  cord  itself  and  extra  medullary  tumors 
which  compress  the  spinal  cord;  the  last  are  again  divided  into  extradural  and 
intradural  neoplasms.  Of  growths  within  the  cord  itself,  glioma,  sarcoma, 
tubercle  and  gumma  are  particularly  found ;  the  intradural  growths  are  chiefly 
fibroma  and  fibro-sarcoma  (usually  pedunculated),  attached  to  a  vessel.  Less 
frequently  myxoma,  psammoma,  teratoma,  lymphangioma ;  while  the  extra- 
dural formations  are  chiefly  lipoma  and  echinococcus  cysts ;  still  Bircher  and  I 
have  described  an  extradural  pedunculated  fibrosarcoma  of  the  cervical  region, 
which  had  passed  out  through  an  intervertebral  foramen  and  had  led  to  the 
formation  of  a  second  tumor  in  the  fossa  supraclaviculars.  The  two  tumors, 
joined  somewhat  like  an  hour  glass,  could  be  extirpated  and  the  patient  cured. 

Etiologically,  we  have  naturally  definite  information  only  about  the  in- 
fectious granulomata  and  the  echinococcus  cysts.  Among  exciting  causes 
trauma  must  sometimes  be  considered;  so,  in  my  case  of  extramedullary  sar- 
coma in  the  upper  lumbar  region  (Fig.  53),  the  patient  had  been  struck  on  the 
back  with  a  cudgel. 

The  development  of  most  cases  of  spinal  cord  tumor  is  marked  by  a  pro- 
dromal stage  with  neuralgiform  pains,  lasting  for  months  or  sometimes  for 
years,  to  be  attributed  to  the  pressure  which  the  tumor  (in  the  great  majority 
of  cases  located  extramedullarily  and  on  the  dorso-lateral  surface  of  the  spinal 


TRANSVERSE     LESIONS    OF    THE     SPINAL    CORD 


161 


cord  and  growing  very  slowly)  exerts  upon  the  posterior  roots.  Later  the 
symptom-complex  of  transverse  lesion  usually  develops  and  increases  very 
slowly,  the  spastic  paresis  occurring  early,  the  disturbances  of  sensibility  later. 
These  last  are  first  manifested  as  hypesthesia  of  the  feet,  and  then  gradually 
ascend  until  an  upper  limit  corresponding  to  the  seat  of  the  tumor  is  reached. 
Then,  while  the  upper  boundary  remains  fixed,  the  intensity  of  the  sensory  dis- 
turbances increases,  eventually  to  complete  anesthesia.  Finally,  disturbances 
of  the  sphincters  are  added;  the  legs  can  be  drawn  up  in  contracture  as  Fig. 
53  shows.  Almost  always  the  Broicn-Sequard  symptom-complex  is  present  for 
some  time  in  typical,  or  more  or  less  modified  development.  Earlier  or  later, 
there  are  also  atrophic  paralyses  of  certain  groups  of  muscles ;  for  example,  of 
the  small  muscles  of  the  hand,  in  tumors  of  the  lower  cervical  region. 

Since  the  treatment  (except  in  the  case  of  the  gummata  which  can  be  cured 
by  antisyphilitic  measures)  can  only  be  surgical,  exact  localization  of  the  tumor 
has,  above  everything  else,  great  importance.     Into  the  points  of  view  important 


Fig.  53. 

Paraplegia,  with  Extreme  Contracture  in  Flexion,  in  Extra-medullary  Fibro-sarcoma 

of  the  Lumbar   Region. 


to  this  end  we  cannot  enter  here;  I  would  refer  you  to  my  "Compendium  of 
Topical  Brain  and  Spinal  Cord  Diagnosis,''  where  this  matter,  which  is  too 
extensive  for  the  scope  of  these  lectures,  receives  a  treatment  sufficiently  ade- 
quate for  practical  purposes.  With  very  rare  exceptions  the  intramedullary 
tumors  present  no  indication  for  surgical  intervention;  not  so  the  extramedul- 
l.irv,  whose  extirpation  presents  no  great  difficulties  to  modern  surgical  tech- 
nique. It  is  noteworthy  that  from  a  clinical  point  of  view  all  extramedullary 
tumors  of  the  spinal  cord,  even  the  sarcomata,  are  to  be  considered  benign, 
since  after  total  extirpation  no  recurrence  has  so  far  been  observed.  The  opera- 
tion is  to  be  considered  as  dangerous,  both  in  the  upper  part  of  the  cervical 
region  and  (on  account  of  the  danger  of  an  infection  of  the  hematoma)  in  the 
lower  part  of  the  spinal  cord.  The  best  outlook  is  furnished  by  tumors  in  the 
lower  cervical  and  upper  dorsal  regions. 

Prom  the  point  of  view  of  differential  diagnosis,  firsl   "pseudo-tumor"  is 


162  LECTURE    X 

to  be  considered,  which,  however,  is  only  to  be  diagnosed  by  "autopsia  in  vivo," 
that  is,  by  opening  the  vertebral  canal,  exploratory  laminectomy.  These  cases 
are  due  to  circumscribed  cyst  formations  upon  the  basis  of  a  localized  serous 
meningitis  which  leads  to  local  compression  of  the  spinal  cord  in  exactly  the 
same  manner  as  true  tumor  formations ;  further,  must  be  mentioned,  multiple 
sclerosis,  a  failure  to  recognize  which,  has  led  to  many  unnecessary  operations. 
In  order  to  protect  yourselves  as  far  as  possible  from  such  a  serious  error,  take 
to  heart  the  following  rules:  Multiple  sclerosis  develops,  as  a  rule,  slower  than 
a  neoplasm  and  often  shows  decided  remissions  in  its  course,  which,  in  the 
clinical  picture  of  tumor  are  either  absent  or  are  of  very  short  duration.  By 
closer  analysis  of  the  pain  phenomena  of  which  patients  with  multiple  sclerosis 
complain,  it  is  usually  found  that  there  is  only  very  annoying  paresthesia  (a 
feeling  of  burning,  of  tension,  of  weight,  of  twitching).  However,  actual  pains 
of  severe,  lancinating  or  pseudo-neuralgic  character  which  are  often  plainly 
dependent  upon  the  weather,  do  also  occur.  Pain  in  the  joints  speaks  for 
multiple  sclerosis.  Disturbances  of  sensibility,  which  are  sharply  limited  above, 
occur  also  in  multiple  sclerosis  occasionally,  but  there  is  never  complete  an- 
esthesia, as  is  frequently  the  case  in  the  advanced  stages  of  a  tumor.  The 
Broum-Scquarcl  topography  of  disturbances  is  very  unusual  in  multiple  sclerosis. 
Of  the  greatest  prognostic  importance  is  also  the  (often  excessively  diffi-» 
cult)  distinction  between  extra-  and  intra-medullary  tumors  in  which  some- 
times exploratory  laminectomy  alone  can  decide.  In  general,  however,  we 
have  the  following  criteria  (of  which,  observe,  no  one  is  pathognomonic):  In 
favor  of  extramedullary  location:  1.  Slow  development  of  the  motor  and  sen- 
sory symptoms.  2.  Tendency  of  the  motor  and  sensory  disturbances  in  spite 
of  increasing  intensity  to  remain  stationary  for  a  long  time  with  regard  to  their 
extent  upward.  3.  Long  duration  of  the  Brozon-Sequard  syndrome.  4.  The 
pseudo-neuralgic  prodromal  stage.  5.  Considerable  intensity  of  the  spastic 
phenomena,  which  persists  even  after  the  occurrence  of  complete  paraplegia. 
6.  The  presence  of  motor  irritative  phenomena,  spasms,  twitching.  7.  Slight 
development  of  degenerative-atrophic  paralyses,  striking  lack  of  relation  be- 
tween the  muscular  atrophy  and  the  only  slight  changes  of  electric  irritability. 
8.  Sensitiveness  to  pressure  over  the  spinal  column.  9.  Yellow  color  (xantho- 
chromia) and  increase  of  albumin  in  the  cerebro-spinal  fluid.  On  the  other 
hand  a  typical  and  marked  dissociated  anesthesia  speaks  strongly  for  the  as- 
sumption of  an  intramedullary  tumor,  while  a  simple  prevalence  of  disturbance 
of  the  pain  and  temperature  sense  as  compared  to  that  of  the  other  senses  also 
is  not  rare  in  extramedullary  tumors  (great  sensitiveness  of  the  pain  and 
temperature  fibers  to  pressure). 

4.     LESIONS   OF   THE    SPINAL   CORD    DUE    TO   AFFECTIONS    OF 
THE  AERTEBRAL  COLUMN 

We  will  only  consider  this  subject  quite  cursorily,  selecting  what  is  im- 
portant neurologically ;  the  surgical  side  of  the  matter  does  not  come  within 
my  province. 

Luxations  and  fractures  of  the  vertebral  column  can  lead  to  sudden  "pres- 


TRANSVERSE    LESIONS    OF    THE    SPINAL    CORD  163 

sure  paralysis"  of  the  spinal  cord  or  to  complete  compression  or  rupture  of 
this  latter.  Luxations  are  more  frequent  in  the  cervical  region  and  occur  par- 
ticularly between  the  1st  and  2d  or  between  the  5th  and  6th  cervical  vertebrae. 
Fractures,  on  the  other  hand,  are  strikingly  more  frequent  in  the  lowest  dorsal 
and  upper  lumbar  region.  The  prognosis  depends  particularly  upon  the  to- 
pography of  the  injur}-;  if  this  has  affected  the  upper  cervical  region  death  is 
usually  instantaneous  ("broken  neck"),  but  even  in  the  less  fatal  location  of  the 
lesion,  bed-sores,  paralysis  of  the  bladder,  cystitis,  etc.,  present  such  great 
dangers  that  death  can  occur  even  weeks  later. 

Tumors  of  the  vertebral  column  (above  everything  the  dreaded  cancer 
metastases  after  carcinoma  of  the  breast,  the  uterus,  the  prostate,  etc.),  as 
well  as  Potts'  disease  (spondylitis  tuberculosa)  have,  as  a  result,  slow  com- 
pression of  the  spinal  cord.  The  clinical,  neurological  symptoms  agree  quite 
well  in  both  processes.  The  picture  is  usually  ushered  in  by  shooting  and  tear- 
ing "root-pains,"  partly  in  girdle  form,  partly  radiating  into  the  legs,  and  of 
great  intensity.  Here  the  neuralgiform  prodromal  stage  is,  as  a  rule,  of  much 
shorter  duration  than  in  tumors  of  the  spinal  cord.  Later,  band-like  hypes- 
thetic  zones  usually  develop,  often,  also  (as  the  result  of  compression  of  the 
anterior  spinal  roots),  atrophic  paralyses  of  certain  groups  of  muscles  corre-. 
sponding  to  the  location  of  the  pressure.  An  increasing  exaggeration  of  the 
tendon  reflexes  in  the  leg,  the  appearance  of  the  Babinski,  Oppenheim,  and 
Mendel-Bechterew  reflexes,  ankle  clonus,  etc.,  are  the  threatening  forerunners 
of  paraplegia,  which,  ushered  in  with  increasing  weakness  of  the  legs,  occurs  at 
tin-  start  without  hypertonia  of  the  muscles,  but  later,  however,  can  develop 
into  marked  spastic  paresis.  The  tuberculous  caries  of  the  upper  cervical 
vertebrw  and  of  the  atlanto-occipital  joint  which  occurs  both  in  children  and  in 
adults,  presents  a  particularly  typical  picture,  with  which  it  is  necessary  to  be 
acquainted,  since  here  the  "gibbus"  which  occurs  in  other  locations  is  absent. 
The  prodromal  symptoms  consist  in  stiffness  of  the  neck  and  bilateral  neuralgia 
of  the  occipital  nerve,  also  in  "Rust's  phenomenon"  in  which  the  patient  on 
changing  his  position  supports  his  head  with  his  hand.  Oppenheim  has  also 
observed  unilateral  atrophy  of  the  tongue  and  spinal  accessory  paralysis. 
Later,  paralytic  symptoms  of  motor  and  sensory  nature  appear,  both  reaching 
up  to  the  neck. 

As  to  the  treatment  of  diseases  and  injuries  of  the  vertebral  column  I  would 
tefer  to  the  surgical  text-books.  As  a  palliative,  "posterior  radicotomy"  (par- 
ticularly dangerous  lure,  however)  in  the  affected  region  conies  into  question. 
To  the  treatment  of  the  nervous  symptoms  remaining  after  cure  of  the  causal 
lesions,  the  principles  already  repeatedly  laid  down  apply. 


LECTURE    XI 

The  Syphilogenic  Diseases  of  the  Central  Nervous 

System 

As  syphilogenic  we  denominate  those  lesions  of  the  brain  and  spinal  cord, 
for  whose  origin  syphilitic  disease  in  the  affected  individual  is  a  prerequisite. 
Among'  these  syphilogenic  organopathics  we  must  separate  two  categories  from 
one  another:  1,  the  syphilitic,  and  2,  the  meta-  or  para-syphilitic  affections. 
In  the  first,  there  is  development  of  the  pathologico-anatomical  changes  in 
general  characteristic  for  syphilis,  in  the  region  of  the  nerve  centers ;  in  the 
last,  however,  there  are  degenerative  lesions  of  definite  portions  of  the  cerebro- 
spinal apparatus,  which  histologically  have  nothing  in  common  with  the  ter- 
tiary manifestations  as  we  find  them  in  other  organs.  Since  now  we  will  save 
true  "lues  cerebro-spinalis"  for  a  later  lecture,  in  the  description  of  the  meta- 
syphilitic  nervous  diseases,  we  will  start  with  the  so   common   tabes  dorsalis. 

The  study  of  this  will  give  us  the  opportunity  of  touching  upon  many 
points,  which  apply  also  to  the  other  metasyphilitic  affection  of  the  central 
nervous  system,  to  progressive  paralysis,  in  the  description  of  which  we  will 
hence  be  in  a  position  to  express  ourselves  somewhat  more  briefly. 

A.     Tabes  Dorsalis 

By  this  name,  after  Romberg's  example,  is  quite  generally  denominated  a 
disease  for  which  Duchennc,  of  Boulogne,  to  whom  we  owe  the  first  satisfactory 
description  and  nosological  separation  of  the  disease  picture  in  question,  in 
1858,  had  proposed  the  name  "progressive  locomotor  ataxia";  only  in  France 
is  this  last  expression  still  used  occasionally. 

The  syphilogenic  nature  of  tabes  is,  on  the  other  hand,  a  discovery  of  the 
last  two  decades  which  we  owe  particularly  to  the  investigations  of  Fournicr 
and  of  Erb.  In  his  latest  comprehensive  statistics  from  the  year  1901,  Fournicr 
was  able  to  demonstrate,  positively,  a  former  syphilitic  infection  in  93  per 
cent,  of  tabetics ;  since  then  we  have  learned  that  also  in  tabetics  who  neither 
give  the  history  of  lues  nor  upon  the  most  exact  physical  examination  present 
residua  of  such  a  disease  (scars,  leucoplakia,  glandular  enlargements,  etc.), 
serological  recognition  of  syphilis  is  not  rarely  to  be  obtained  (through  the 
"Wassermann  reaction"  to  be  mentioned  later).  Even  where  this  reaction  is 
not  positive,  the  possibility  of  a  latent  or  hereditary  syphilis  is  in  no  way  to 
be  excluded.  Mendel  and  Tobias,  in  their  cases  of  tabes  in  virgins,  have  been 
able  with  all  certainty  to  demonstrate  either  a  direct  tabetic  heredity  or  an  ac- 
quired extragenital  infection.     To  the  very  rare  cases  in  which  a  tabetic  has 

164 


SYPHILOGEXIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     165 

recognizably  acquired  syTphilis,  any  value  as  disproving  the  statement  formulated 
by  Mobhis,  "Nulla  tabes  sine  syphili,"  is  to  be  denied.  Hence  I  would  not 
hesitate  to  denominate  a  case  presenting  the  clinical  picture  of  tabes  dorsalis, 
in  which,  however,  acquired  or  hereditary  syphilis  is  with  certainty  to  be  ex- 
cluded as  a  "pseudo-tabes"  (such  a  case,  in  fact,  I  never  yet  observed).  To 
the  conception  of  tabes  belongs,  according  to  our  present  view,  along  with  the 
symptomatological  criteria,  the  causal  connection  with  syphilitic  disease. 

Now,  however,  arises  the  following  question :  "Why  is  tabes  such  a  rare 
result  of  syphilis"?  {llcumont  has,  for  example,  shown  that  among  3,600 
syphilitica  only  40,  that  is,  1.1  per  cent.,  later  became  tabetic.)  May  not 
other  factors  be  determining  for  the  development  of  the  disease  (usually  5  to 
15  years  after  the  infection)  ? 

In  fact,  the  opinion  is  wide-spread  that  here  neuropathic  heredity  plays  a 
part.  Erb  has  found  in  28  per  cent,  of  his  tabetics  hereditary  neuropathic 
factors;  in  42  per  cent,  individual  nervous  disturbances — not  belonging  to 
tabes.  If  tabetic  patients  are  systematically  investigated  as  to  the  so-called 
'"stigmata  of  degeneration"  (malformations  of  the  skull,  of  the  palate,  of  the 
ears  "h'ssural  angiomata,"  etc.),  these  are  found  more  frequently  and  in  the 
average  in  richer  combination  than  in  healthy  persons.  For  the  view  that  an 
originally  defective  structure  of  the  spinal  cord  can  predispose  this  organ  to 
the  development  of  tabes  in  case  of  syphilitic  infection,  the  circumstance  that 
it  is  in  tabetics  that  most  of  the  cases  of  heterotopia  and  abnormal  course  of 
the  fibers  have  been  described,  is  of  some  importance. 

Along  with  reduced  power  of  resistance  (which  in  the  origin  of  progressive 
paralysis  appears  to  play  the  same  role  as  in  that  of  tabes)  differences  in  the 
luetic  virus  also  are  probably  determining  factors  in  the  later  involvement  of 
the  central  nervous  system.  There  are  apparently  definite  strains  of  spiro- 
chietae  whose  toxines  possess  particularly  strong  neutropic  properties — a  "Lues 
nervosa,"  a  "Syphilis  a  virus  nerveux."  We  are  forced  to  take  this  point 
of  view  by  the  numerous  observations,  in  which,  after  the  luetic  infection  of 
groups  of  people  from  a  recognizable  common  source,  syphilogenic  diseases  have 
later  occurred  in  all  or  in  a  large  number  of  those  infected.  Erb,  for  example, 
mowed  that  4  men  infected  from  the  same  prostitute  later  all  became  tabetic 
or  paralytic.  Broslus  saw,  12  years  later,  5  of  7  victims  of  an  epidemic  of 
syphilis  among  glass-blowers;  4  of  these  were  tabetic  or  paralytic.  In  an 
entirely  analogous  manner  must  be  interpreted  the  considerable  number  of 
recognized  cases  of  family  tabes  or  general  paresis,  in  which  father  or  mother, 
or  both  parents,  along  with  one  or  several  children,  are  afflicted  with  tabes. 
Family  predisposition  cannot  always  be  invoked  here,  since,  as  a  rule,  the 
parents  are  not  consanguine.  As  group  diseases  in  a  small  way,  the  not  at 
nil  infrequent  eases  of  conjugal  Jabes  or  paresis  can  be  subjected  to  the  same 
method  of  consideration. 

An  important  question  is  whether  the  insufficiently  treated  syphilitic  runs 
a  greater  risk  of  later  metaluetic  disease  than  one  subjected  to  adequate 
treatment.  Many  authors,  particularly  syphilologists,  have  decided  this  ques- 
tion in  the  affirmative,  while,  on  the  other  hand,  there  are  those  who  would 
make  mercurial   intoxication  in  part   responsible  for  the  origin  of  tabes  and 


166  LECTURE    XI 

paresis.  According  to  my  idea,  both  views  are  incorrect.  What  could  be  more 
conclusive  than  conjugal  cases  of  tabes  in  which  we  hear  that  one  of  the  pair 
has  had  extended  anti-syphilitic  treatment,  the  other  none  at  all,  and  where 
still  both  have  equally  acquired  tabes?  We  will  occupy  ourselves  more  ex- 
tensively with  this  question  in  the  next  lecture. 

Edinger  has  taken  a  totally  different  standpoint,  in  that  he  attributes  to 
functional  use  (that  is,  to  the  measure  of  work  required  from  the  metaluetic 
spinal  cord)  a  considerable  role  in  the  origin  of  tabes.  In  many  cases  this  view 
has  much  that  is  illuminating,  namely,  in  those  in  which  tabes  begins  quite 
acutely  after  severe  bodily  excesses*  or  the  first  symptoms  are  noted  in  an 
extremity  continually  used.  Not  rarely,  however,  Edinger's  "overuse  theory" 
fails  us,  and  we  know  tabetics  enough  whose  disease  remains  particularly  mild 
and  stationary,  although  they,  in  spite  of  all  medical  advice,  continually  over- 
exert themselves  (for  example,  as  hunters  and  riders). 

Probably  race  predisposition  comes  also  into  question  in  the  tendency  of 
syphilitics  to  develop  metaluetic  nervous  diseases.  It  has  been  scientifically 
demonstrated  that  in  many  races  saturated  with  syphilis,  tabes  either  does 
not  occur  or  is  extraordinarily  rare  (for  example,  among  the  Kurghise  of 
Central  Asia,  the  negroes  and  mulattoes  of  North  America,  the  Arabs  of 
Algeria,  etc.f  Finally,  as  occasional  accessory  factors,  getting  chilled  or 
wet  and  trauma  should  be  mentioned,  which,  along  with  overexertion,  explains 
the  fact  that  after  campaigns  an  increase  in  the  number  of  cases  of  tabes 
has  been  repeatedly  observed  among  the  combatants. 

Pathological  Anatomy 

Tabes  is  pathologico-anatomically  a  systematic  degeneration  in  the  region 
of  the  posterior  roots  of  the  spinal  cord. 

We  call  systematic,  as  already  indicated  (page  111),  those  diseases  of  the 
spinal  cord  whose  lesions  are  limited  to  definite  fiber  systems  (as,  for  example, 
amyotrophic  lateral  sclerosis  and  tabes),  while  "asystematic"  or  "diffuse" 
affections  are  those  whose  anatomical  substratum  is  not  limited  to  definite 
neurons  (as  multiple  sclerosis  and  tumors  of  the  spinal  cord). 

A  knowledge  of  the  structure  of  the  posterior  spinal  roots  and  of  the 
further  course  of  their  fibers  is  of  great  importance  for  the  understanding  of 
the  pathological  anatomy  of  tabes  dorsalis. 

The  fibers  of  the  posterior  roots  have  their  cells  of  origin  in  the  spinal 
ganglia;  these  cells  of  origin,  through  one  of  their  processes  which  runs  in 
the  course  of  a  nerve  trunk,  are  in  relation  with  the  different  peripheral  appa- 
ratus, which  we  find  partly  as  free  nerve  endings,  partly  as  terminal  bodies 
(touch  cells  and  bulbs,  the  Vater-Pacini  corpuscles,  etc.)  in  the  integument, 
the  mucous  membranes,  the  mesentery,  the  joint  surfaces,  etc.  The  other  or 
central  process  of  the  spinal  ganglion  cell,  however,  enters  the  spinal  cord 
through  a  posterior  root. 

*  Corresponding  to  a  certain  extent  to  the  generally  recognized  predisposing  role  which 
mental  overexertion,  excitement,  etc.,  play  in  the  causation  of  progressive  paralysis. 

f  This  statement  is  incorrect  in  so  far  as  it  relates  to  the  American  negro. — Translator. 


SYPHILOGEMC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     167 

The  posterior  root  fibers,  however,  we  separate  into  different  categories, 
according  to  their  further  course.  The  "short  fibers"  pass  directly  through 
the  rim  zone  of  the  posterior  horn  into  the  gray  matter  and  break  up  either 
about  the  cells  of  the  anterior  horn,  or  about  those  of  the  posterior  horn,  on 
the  same  side  of  the  cord.     In  the  first  instance  they  serve  to  convey  reflex 


=  Short  fibers. 


Kl  ) 

M  =  Fibers  of  medium  length. 
L  =  Long  fibers. 


The  Different  Categories  of  Fibers  in  the  Posterior  Roots  and  Their  Continuation 
in  the  Spinal  Cord. 

(i.  It.  —.  I  Join  r'.i  tract.  Kh.  S.  St.        Direct  cerebellar  tract. 

II.  St.       Posterior  column.  I'v.  S.  St.  =  Lateral  pyramidal  tract, 

l'y.  V.  St.  =  Anterior  pyramidaj  tract. 


stimuli  to  the  anterior  roots,  in  the  second  to  conduct  pain,  temperature  and 
touch  stimuli  to  the  so-called  "Tractus  spino  thalamicus,"  which,  after  cross- 
ing to  the  other  side  of  the  cord,  conducts  these  sensations  toward  the  brain. 
The  "middle  fibers"  of  the  posterior  roots  pass  through  the  posterior  roots 
on  into  the  posterior  columns,  and   from   there  into  the  base  of  the  posterior 


168 


LECTURE    XI 


horn  on  the  same  side,  and  end  about  the  cells  of  the  column  of  Clarke,  from 
which  the  stimuli  transmitted  to  the  cord  through  this  set  of  neurones  are 
carried  in  the  direct  cerebellar  tract  to  the  cerebellum.  The  third  variety, 
the  "long  fibers"  of  the  posterior  roots  also  enter  the  posterior  columns  through 


Fie;.  55. 
Structure  of  the  Posterior  Columns. 

G — Column  of  Goll  (Funiculus  gracilis). 
B — Column  of  Burdnch  (Funiculus  cuneatus). 

a — Long  fillers  of  the  posterior  roots  from  the  upper  half  of  the  body, 
b — Long  fibers  of  the  posterior  roots  from  the  lower  half  of  the  body. 


the  posterior  root  zone,  then  run  in  these  columns  upward,  and  end  in  the 
"nuclei  of  the  posterior  columns"  in  the  medulla,  from  whence  the  conduction 
of  stimuli  is  transmitted  via  the  optic  thalamus  to  the  opposite  side  of  the 
cerebrum. 

The  fibers  of  the  posterior  columns  as  well  as  those  of  the  direct  cerebellar 


SYPHILOGEXIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     169 

tracts  conduct  "deep  sensibility,"  with  the  difference,  however,  that  the  spino- 
cerebellar tracts  serve  to  transmit  subconscious  perceptions  (of  tactile  and 
coordinatory  nature),  while  the  posterior  column,  along  with  the  same  func- 
tion, also  serves  to  carry  the  conscious  perceptions  of  position  sense  and 
movement  sense  to  the  cerebrum. 

The  following  anatomical  peculiarities  of  the  fibers  of  the  posterior  columns 
should  also  be  noted:    1.   While  the  long  fibers  entering  the  posterior  column 


FlO.  56. 
Lumbar  Tabes.     (Cervical  Region.)      Weigert-Pal  Stain. 


through  a  definite  posterior  root  run  upward,  they  are  continually  forced 
more  and  more  toward  the  median  line  by  fibers  entering  higher  segments 
through  the  posterior  root  zone.  On  account  of  this,  for  example,  in  a  cross 
Bection  through  the  cervical  region,  the  fibers  of  sacral  origin  lie  next  to  the 
septum,  those  from  the  lumbar  region  farther  out,  then  the  dorsal  fibers,  and 
finally,   next  to  the  posterior  horn,  those  of  the  cervical  neurones. 

In  the  cervical  cord,  however,  a  macroscopical  separation  has  occurred  in 
this  way  between  flic  long  fibers  of  the  posterior  roots  which  come  from  the 
tower  half  of  the  body  (thai  is,  from  the  4th  thoracic  segment  downward), 
which  are  united  in  the  columns  of  (loll,  and  those  from  the  upper  half  of  the 
body  (that  is,  from  the  4th  thoracic  segment  upward)  which  make  up  the 
column  of  Burdach   (sec  Fig.  55). 

2.  Each  one  of  tin'  long  fibers  of  the  posterior  roots,  before  it  turns 
upward  in  the  posterior  columns,  gives  "If  a  branch  (a  collateral)  which  de- 
scends  some  distance,  also  in   the  posterior  column.      (In  Figs.  54  and   55   tin- 


170  LECTURE    XI 

collaterals  are  omitted  for  the  sake  of  simplicity.)  These  descending  elements 
of  the  posterior  root  system  lie  together  in  a  definite  bundle,  the  so-called 
"Schultze's  comma"  (see  above  Fig.  52,  page  158);  this  last,  for  example, 
in  the  upper  lumbar  segments  contains  no  lumbar  fibers,  but  those  which 
have  come  in  at  a  higher  level  of  the  spinal  cord,  that  is,  in  the  instance  selected, 
belong  to  the  dorsal  segments. 

3.  The  long  fibers  of  the  posterior  root  system  are  not  the  only  nervous 
structures  of  the  posterior  column  area.  Rather  are  there  mixed  among  them 
the  so-called  "endogenic"  fibers  of  the  posterior  column,  the  so-called  inter- 
segmental or  association  tracts,  which  connect  with  one  another  different 
levels  of  the  posterior  horns.  At  certain  places  in  the  posterior  column  area 
the  endogenic  fibers  lie  so  close  together  that  on  cross  sections  the  long  fibers 
of  the  posterior  roots  appear  quite  thinned  out.     These  "endogenic  fields  of  the 


Fi„.  57. 
Lumbar  Tabes.     (Dorsal  Region.)      Weigert-Pal  Stain. 

posterior  columns"  are,  in  the  neighborhood  of  the  commissure,  the  "ventral 
field  of  the  posterior  column"  (Zona  cornu-commissuralis),  at  the  periphery 
of  the  spinal  cord,  along  the  septum,  the  "median  peripheral  field  of  the 
posterior  column"  (Zona  septo-marginalis). 

Since  now  tabes,  in  the  great  majority  of  cases,  begins  as  "tabes  inferior," 
that  is,  as  degeneration  of  the  posterior  roots  in  the  lumbar  region  ;  after  the 
above  remarks  it  is  understandable  that  in  the  early  stages  of  the  disease, 
in  sections  through  the  higher  levels  of  the  spinal  cord,  it  is  only  the  mesially 
placed  long  tracts  of  the  posterior  column,  that  is,  those  which  have  come 
up  from  the  lumbar  region;  in  other  words,  the  columns  of  Goll,  which  are 
degenerated  (see  Figs.  56  and  57).  In  sections  through  the  lumbar  segments, 
however,  a  degeneration  involving  also  the  lateral  portions  of  the  posterior 
column  is  found,  indeed,  it  is  most  marked  in  the  region  of  the  posterior  root 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     171 

zone  (see  Fig.  58).  Upon  careful  microscopical  examination  it  is  found  fur- 
ther, that  "Schultze's  comma"  is  not  affected  by  the  degeneration.  This 
is  not  remarkable,  since  its  fibers  come  from  the  higher  roots  not  yet  dis- 
eased. The  "endogenic  fields  of  the  posterior  column"  are  in  contrast  with 
the  others  by  being  relatively  intact,  since  they  carry  a  large  number  of  fibers 
which  have  nothing  to  do  with  the  posterior  root  system.  In  advanced  cases 
of  tabes  on  account  of  the  involvement  of  higher  segments  of  the  cord  in  the 
disease  process,  "Schultze's  comma"  degenerates  also,  and  finally  even  in  the 
cervical  region   all   the  posterior  area  is  involved. 

The   central    point    in   tabetic   disease   of   the    spinal    cord   is   the   primary 
degeneration   of   peripheral   sensory   neurones.      In   this    there   remain   longest 


'/ 


Fig.  5S. 
(Lumbar  Region.)      Weigert-Pal  Stain. 


intact  (at  least,  according  to  external  appearance)  the  cells  themselves,  that 
is,  those  of  the  spina]  ganglia,  while  degeneration  shows  itself  first  in  their 
central  processes  (posterior  roots  and  posterior  columns).  Their  peripheral 
processes  (which  are  to  be  sought  in  the  sensory  nerves)  usually  are  only 
found  altered  in  advanced  stages  of  the  disease.  That  the  radicular  and 
medullary  part  of  this  neurone  is  more  intensely  affected  than  the  peripheral 
bar!  is  partially  explained  by  the  following  discovery  of  Obersteiner  and 
Redlich.  These  authors  showed,  namely,  that  at  the  point  of  entrance  of 
the  posterior  roots  into  the  spinal  cord  there  is  normally  a  sort  of  strangula- 
tion of  the  root  bundle,  since  at  this  point  the  pia  is  thickened  and  is  covered 
with  a  particularly  close  layer  of  glia.  This  circumscribed  part  of  the  pos- 
terior root   forms  evidently   a  "Locus  minoris   rcsistentia?."     On  this  account, 


172  LECTURE    XI 

even  in  incipient  tabes,  it  is  in  the  lumbar  region  where  these  constrictions 
are  most  marked  that  the  roots  are  earliest  and  most  intensely  affected.  The 
whole  sensory  neurone  of  the  first  order  seems  on  account  of  the  method  of 
development  peculiar  to  it  (the  sensory  nerves  grow  in  ontogenesis  only  sec- 
ondarily into  the  spinal  cord)  to  have  become  the  specially  vulnerable  part  of 
the  central  nervous  system.  A  certain  specific  affinity  of  metasyphilitic  toxines 
for  this  system  of  fibers  (also  for  the  others  affected  by  tabes,  e.g.,  the  optic 
nerve)  seems  probable,  indeed. 

Symptomatology 

As  we  now  proceed  to  the  consideration  of  the  manifold  and  interesting 
symptomatology  of  tabes  dorsalis,  regardless  of  the  different  clinical  pictures 
which  it  can  present  to  us,  we  will  next  pass  in  review  all  its  disease  symptoms 
in  as  rational  grouping  as  possible.  We  will  begin,  of  course,  with  those 
symptoms  which  can  be  directly  attributed  to  the  characteristic  anatomical 
findings  in  the  posterior  root  affection. 

This  posterior  root  syndrome  is  made  up  of  the  following  components: 

1.    ATAXIA 

We  have  already  learned  in  the  first  lecture  that  from  disturbance  of  such 
centripetal  impulses  as  inform  us  at  any  given  movement  of  the  position  of 
our  limbs,  the  clinical  phenomena  of  ataxia  or  disturbance  of  coordination 
results. 

The  ataxia  of  the  tabetic  produces  an  exceedingly  typical  disturbance  of 
gait,  hence  the  name  "progressive  locomotor  ataxia"  which  Diichenne,  of 
Boulogne,  applied  to  this  whole  nosological  unit.  There  is  a  "dysmetria"  in 
the  movements  necessary  for  progression,  in  that  the  legs  are  swung  out  and 
shoot  forward  beyond  the  measure  of  a  normal  step;  further,  the  steps  are  of 
unequal  length.  We  notice  also  abnormal  positions  of  the  joints  during 
locomotion,  so  that,  for  example,  the  swung  leg  remains  strongly  extended 
at  the  knee,  the  point  of  the  foot  is  directed  too  much  outward  and  the  foot 
strikes  the  ground  not  with  the  ball,  but  with  the  heel.  In  the  most  extreme 
degrees  of  tabetic  ataxia  walking  becomes  impossible,  even  when  the  patient 
is  supported  on  both  sides,  as  every  attempt  to  make  steps  degenerates  into  a 
series  of  contrary  movements  in  the  lower  extremities. 

On  the  other  hand,  in  the  early  stages  of  tabes,  the  incoordination  of  the 
locomotor  mechanisms  is  not  to  be  demonstrated  without  special  tests.  Some 
easily  applicable  tests  adapted  to  bring  out  an  ataxia  not  evident  in  ordinary 
locomotion  are  walking  on  a  straight  line  (in  which  each  foot  is  alternately 
to  be  placed  exactly  in  front  of  the  other),  quick  turning  about  at  command, 
walking  on  the  tips  of  the  toes  or  with  the  knees  bent,  walking  backward, 
walking  up  and  especially  downstairs,  finally  walking  with  closed  eyes. 

This  last  test  makes  evident  to  a  certain  extent  the  compensating  influence 
which  the  optical  control  of  movements  can  exercise  upon  the  coordination 
of   these   last   in   tabetic   patients ;   also    walking   in   the   dark   is    considerably 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     173 

more  difficult  for  them.  This  influence,  however,  makes  itself  felt  not  only 
in  relation  to  locomotor  or  dynamic,  but  also  in  static  ataxia.  Upon  this  de- 
pends the  well-known  Romberg's  symptom;  if  a  tabetic  is  asked  to  stand  with 
his  feet  pressed  close  together  (that  is,  his  base  of  support  is  made  as  small 
as  possible),  and  then  to  close  his  eyes,-  he  immediately  begins  to  stagger  and 
would  fall  without  support.  Where  in  light  or  incipient  cases  Romberg's 
phenomenon  is  not  present  or  is  imperfect,  it  is  well  to  test  coordination  by 
having  the  patient  stand  on  one  leg.  It  is  then  noticed  that  where  this  last 
is  disturbed,  the  patient  is  unable  to  balance  himself  on  one  leg  when  he 
closes  his  eyes,  often  even  with  open  eyes.  In  advanced  stages  of  the  disease 
static  ataxia  often  appears  spontaneously  in  that  the  body  of  the  patient, 
when  he  is  sitting  upright,  sways  continually  from  side  to  side. 

Tests  for  demonstrating  ataxia  of  the  legs  when  the  patient  is  lying  down 
are  the  following:  He  is  asked  to  describe  a  circle  with  each  foot;  to  touch 
an  object  held  before  him  with  his  toe;  to  touch  one  knee  with  the  opposite 
heel  (first  with  open  and  then  with  closed  eyes).  For  testing  for  ataxia  in 
the  upper  extremities  the  finger-finger  and  finger-nose  tests,  in  which  the 
patient  first  with  open  and  then  with  closed  eyes  brings  his  index  fingers  to- 
gether in  front  of  him,  or  touches  the  point  of  his  nose,  are  customary. 

2.  HYPOTONIA 

Since  for  the  preservation  of  tonus  a  continual  passage  of  stimuli  from 
the  posterior  roots*  to  the  motor  cells  of  the  anterior  horn  is  necessary,  it 
is  not  remarkable  that  the  prevention  of  this  passage  by  the  tabetic  degen- 
eration of  the  posterior  roots  leads  to  an  abnormal  relaxation  of  the  muscles. 
In  the  region  of  the  pelvis  and  of  the  lower  limbs  the  hypotonia  is  shown  by 
the  patient  being  able  to  spread  his  legs  abnormally  wide  apart,  or  in  that 
we  can  bring  the  leg,  extended  at  the  knee,  into  an  acute  angle  with  the  trunk, 
and  under  certain  conditions,  indeed,  we  can  in  this  manner,  without  trouble, 
•"make  a  shoulder  of  the  leg" — things  which  the  healthy  person  can  do  only 
al'ti ■]•  long  training  (the  "grand  ecart"  of  the  ballet  dancers,  the  "art"  of 
tli>-  "snake  man").  In  the  knee,  on  account  of  the  relaxation  of  the  biceps, 
(temitendinosus  and  semimembranosus  a  hypotonic  subluxation  backward,  the 
so-called  "Genu  recurvatum"  occurs.  Fig.  59  shows  this  deformity,  which, 
however,  is  not  pathognomonic  for  talus  dorsalis,  hut  can  occur  in  muscular 
flystrophy,  neuritis,  etc.  On  the  other  hand,  hypo  tonus  of  the  quadriceps  some- 
times permits  bringing  the  heel  up  against  the  buttock. 

:i.    AREFLEXIA 

While  in  llic  mtv  first  stages  of  tabes  increase  of  the  tendon  reflexes  is  not 
tare  (evidently  as  a  symptom  of  irritation  of  the  posterior  roots),  in  the 
pourse  of  the  disease  these  reflexes  almosl    always  gradually  disappear.     On 

■  This  is  by  way  of  the  short  posterior  runt  fillers  which  enter  Hie  anterior  horn  directly  and 

hre;ik  up  about  its  motor  cells. 


174- 


LECTURE    XI 


account  of  the  intimate  relation  between  tonus  and  reflexes  the  pathogenesis 
of  this  hyporeflexia  and  areflexia  corresponds  to  that  of  tabetic  muscular 
relaxation  (see  also  Lecture  I,  page  7).  Most  important  among  the  reflex 
anomalies  of  tabes  is  the  so-called  WestphaVs  phenomenon,  the  loss  of  the 
patellar  reflex.  Complete  loss  can  only  be  diagnosed  when  striking  upon  the 
patellar  tendon  produces  no  contraction  in  the  quadriceps  muscle,  all  tension 
of  the  thigh  muscles,  voluntary  or  involuntary,  being  avoided.  An  excellent 
means  of  attaining  the  best  conditions  for  this  experiment  is  furnished  by  the 
so-called  "Jcndrassik's  maneuver";  in  this  the  patient  sits  with  loosely  crossed 

legs  on  a  comfortable  chair,  his  head  thrown 
back,  looking  at  the  ceiling,  and  the  fingers 
of  each  hand  hooked  with  those  of  the  other  ; 
after  the  patient  has  been  previously  in- 
structed to  pull  hard  with  his  hands  as  the 
last  number  is  given,  1 — 2 — 3  is  counted, 
and  immediately,  while  the  patient's  atten- 
tion is  directed  away  from  his  legs,  a  firm 
stroke  is  made  witli  the  percussion  hammer 
upon  the  ligamentum  patella1.  The  patellar 
reflex  can  also  be  tested  while  the  patient 
is  lying  down ;  the  leg  to  be  tested,  the  knee 
bent,  is  lifted  from  the  bed  by  an  aide,  the 
patient  being  entirely  passive  and  relaxing 
his  muscles.  [The  limb  is  best  rotated 
slightly  inward. — Translator.]  Testing  the 
Achilles  tendon  reflex  is  almost  as  important 
as  that  of  the  patella  tendon;  it  is  most 
easilj'  accomplished  if  the  patient  kneels 
upon  a  well-cushioned  chair  and  turns  his 
back  to  the  examiner,  the  feet  hanging  over 
the  edge  of  the  chair.  The  different  tendon 
and  bone  reflexes  in  the  upper  limbs  ( see 
table,  page  8),  usually  after  exaggeration 
at  the  start,  are  lost  later  than  those  of  the 
lower  extremities  (except  in  the  so-called 
tabes  superior). 
Before  reduction  or  loss  of  the  tendon  reflexes  are  interpreted  as  indicating 
tabes  dorsalis,  it  is  necessary  to  find  out  by  questioning  if  the  anomaly  can 
be  brought  into  connection  with  any  former  local  disease;  so,  for  example,  a 
sciatica  very  often  leaves  behind  a  hypo-  or  areflexia  of  the  Achilles  tendon 
on  the  same  side. 

Loss  of  reflex  on  one  side,  or  difference  between  the  reflexes  on  the  right  and 
on  the  left,  are  frequent  symptoms  in  the  course  of  tabes  dorsalis.  In  con- 
tradiction to  the  tendon  reflexes,  the  skin  reflexes  are  almost  always  retained ; 
often,  indeed,  they  are  abnormally  lively. 


Fig.  59. 
Genu  Recurvatum. 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     173 

L    DISTURBANCES   OF   SENSIBILITY 

Even  in  the  carl}'  stages  of  tabes,  disturbances  in  the  power  of  appre- 
ciating different  qualities  of  sensation,  which  in  advance  stages  of  the  dis- 
ease may  increase  to  anesthesia  proper,  make  themselves  felt.  Both  skin  and 
deep  sensibility  may  be  affected  (see  page  6). 

a.  Disturbances  of  Superficial  Sensibility 

As  to  the  location  of  the  hyperesthesias  and  anesthesias  to  be  found  on 
the  skin,  as  a  rule  they  plainly  follow  the  so-called  "radicular  type"  in  that 
the  areas  of  reduced  or  lost  sensation  do  not  agree  with  the  distribution  of 
peripheral  nerves,  but  on  the  extremities  involve  more  or  less  longitudinal 
areas;  on  the  trunk,  circular  zones. 

The  difference  between  "peripheral"  and  "radicular"  topography  of  dis- 
turbances of  sensibility  is  explainable  through  the  following  relations.  Each 
spinal  ganglion  sends  its  peripheral  processes  into  different  sensory  nerves,  each 
sensory  nerve  contains  fibers  which  originate  in  different  spinal  ganglia.  In- 
dependent of  the  often  complicated  paths  which  they  have  followed  in  the 
peripheral  nerve,  at  the  extreme  periphery,  that  is,  on  the  surface  of  the  body, 
the  sensory  fibers  so  arrange  themselves  that  those  arising  from  a  definite 
spinal  root  supply  a  definite  region.  This  region  is  called  a  "radicular  zone" 
or  "root  field."  In  it  the  sensory  fibers  originally  united  in  one  posterior 
root  come  together  again,  even  when  they  are  transmitted  to  the  skin  through 
different  peripheral  nerves.  Figs.  60  and  61  expose  the  difference  between  "root 
fields"  and  peripheral  nerve  areas.  In  them  the  radicular  zones  are  repre- 
sented as  occupying  the  area  on  each  side  of  the  line  which  bears  their  root 
number.  The  root  fields  of  the  individual  posterior  roots  overlap  one  an- 
other somewhat  like  the  tiles  on  a  roof.  The  special  arrangement  of  the 
radicular  zones  in  the  extremities,  in  which  in  contradistinction  to  the  circular 
arrangement  on  the  trunk  a  longitudinally  directed  distribution  prevails,  is 
explainable  from  their  ontogenetic  relations.  In  the  embryo,  the  first  begin- 
nings of  the  limbs,  growing  out  from  the  trunk,  take  with  them  the  "dcrma- 
totomes"  (fetal  skin  segments)  lying  in  their  way  and  corresponding  to  the 
same  segments  of  the  cord,  and  since  the  limbs  grow  out  more  or  less  vertically 
from  the  axis  of  the  trunk,  in  them  the  principle  of  circular  arrangement  is 
not  impaired  by  the  extension  of  the  segmental  distribution  in  the  direction 
of  their  axes   (longitudinally). 

Tin-  radicular  hypesthetic  or  anesthetic  zones  are  found  particularly  fre- 
quently on  the  inside  of  tin  [eg  and  foot  and  on  the  inside  of  the  upper  ex- 
tremities; often,  also,  in  the  form  of  a  girdle  or  a  half  girdle  (since  asymmetry 
is  not  at  all  rare)  they  surround  the  thorax  or  the  epigastrium  (see  Fig.  62). 
In  the  early  stages  we  find  sometimes  instead  of  the  more  or  less  bandlike 
hypesthetic    or    anesthetic    zones,    insulated     areas    of    hvpesthesia     and     anes 

thesia,  usually  asymmetrically  distributed.  So  much  for  the  topography  of  the 
tabetic  sensory  disturbances  in  the  integument.  As  to  their  quality,  they 
can  affect  equally  the  tactile,  the  pain  or  the  temperature  senses.      Usually  one 


176  LECTURE    XI 

variety  of  sensation  is  more  affected  than  the  others,  and  this  is  usually 
the  pain  sense.  Apart  from  diminution  of  sense  perception  we  often  find 
delay  in  conduction  in  which  the  patient  first  experiences  pain  some  seconds 
after  a  pin  prick.     If  in  such  cases  tactile  sense  is  undisturbed,  one  prick  can 


produce  two  sensations,  in  that  it  is  experienced  first  as  a  touch,  and  some- 
what later  as  pain.  Further,  the  quite  frequent  faulty  localization  of  touch, 
pain  and  temperature  stimuli  must  be  mentioned;  if  this  is  so  marked  that 
the  patient,  his  eyes  closed,  when  solicited  to  indicate  the  spot  irritated,  mis- 
takes the  side  of  the  body,  that  is,  locates  a  prick  on  the  right  hand  on  the 
left  side,  we  speak  of  "Allocheiria."     Hypesthesia  or  anesthesia  of  the  soles 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     177 

of  the  feet,  a  very  common  tabetic  symptom,  is  usually  experienced  as  very 
disagreeable  by  the  patient  and  impairs  his  power  of  locomotion. 


b.  Disturbances  of  Deep  Sensibility 

Strictly  considered,  ataxia,  already  studied,  belongs  among  the  disturbances 
of  deep  sensibility,  as  shown  in  Lecture  I.  Besides  this,  interference  with 
"bathyesthesia"  is  evident  in  most  cases  of  tabes  after  they  have  reached  a 
certain  degree  of  intensity.  One  can  convince 
himself  of  the  loss  of  "position  sense"  without 
difficulty  if,  having  the  patient  close  his  eyes, 
one  extremity  is  put  in  a  certain  position  and 
the  subject  is  requested  to  put  the  opposite  one 
in  an  exactly  similar  position.  The  gross  errors 
which  he  makes  give  evidence  of  the  fact  that 
he  has  lost  control  over  the  relative  attitude 
of  his  limbs  without  the  assistance  of  the  visual 
sense.  In  analogous  manner  loss  of  ''movement 
sense"  may  be  recognized.  Disturbances  of  the 
position  and  movement  sense  in  the  fingers  lead 
to  "stereo-anesthesia,"  that  is,  inability  to 
recognize  the  form  of  objects  by  handling  them. 
The  testing  of  bone  sensibility  with  a 
tuning-fork  can  render  valuable  service  even  in 
the  early  stage  of  tabes.  The  loss  of  vibration 
sense,  or  its  manifest  reduction,  is,  as  I  have 
recognized,  the  most  delicate  reagent  for  detect- 
ing beginning  lesions  of  the  posterior  columns. 
To  the  early  disturbances  of  deep  sensibility 
usually  belong  also  anesthesias  of  certain  nerve 
trunks  and  viscera.  The  severe  pain  which  firm 
compression  of  the  ulnar  or  peroneal  nerves 
normally  produces  is  no  longer  to  be  elicited 
(Biernacki's  symptom).  The  same  remark 
applies  to  the  exceedingly  unpleasant  sensation 
which  energetic  pinching  of  the  Achilles  tendon 
produces  in  healthy  people;  this  analgesia  of 
the  Achilles  tendon  is  called  Abadie's  tabes 
symptom.  Of  the  frequent  visceral  analgesias  to  pressure  I  would  mention  those 
of  the  eyeball,  the  larynx,  the  trachea,  the  breast,  the  ovary  and  the  testicle. 


Typical  "Radicular"  Disturbances 
of  Sensibility  in  a  Case  of  Tabes 
Dorsalis. 


5.    TROPHIC    DISTl'KHANCES 


We  must  assume  that,  normally,  the  trophic  influence  which  the  cells  of 
i  In  anterior  horn  of  the  spinal  cord  exert  upon  the  bony  skeleton  and  the 
integument  are  stimulated  to  a  certain  extent  reflexlv  through  the  elements 
of  the  posterior   root    system.      In   this  manner  can  be  explained    the   fact    that 


178 


LECTURE    XI 


tabetic  disease  of  the  posterior  roots  can  sometimes  lead  to  trophic  disturb- 
ances in  this  apparatus. 

In  the  skeleton  the  so-called  "tabetic  spontaneous  fractures"  which  depend 
on  pathological  brittleness  of  the  bones  deserve  special  consideration.  They 
occur  from  the  slightest  causes ;  for  example,  I  saw  fracture  of  the  neck  of 
the  femur  from  stepping  down  from  the  pavement ;  fracture  of  the  radius 
from  striking  the  swinging  arm  against  the  back  of  a  chair.  Characteristic 
of  these  fractures,  whose  occurrence  is  sometimes  the  first  symptom  calling 
attention  to  the  presence  of  tabes,  is  their  painlessness,  an  important  evidence 
of  the  already  mentioned  deep  anesthesia.  Repair  of  these  fractures  some- 
times occurs  normally ;  occasionally, 
however,  the  callus  formation  is  de- 
fective, so  that  pseudo-arthroses  oc- 
cur; on  the  other  hand,  an  excessive 
callus  formation  (leading  to  severe 
disturbances  of  function)  has  re- 
peatedly been  observed.  The  X-ray 
examination  of  the  bones  of  tabetics, 
which  tend  to  undergo  spontaneous 
fracture,  shows  thinning  of  their 
compact  layer ;  besides  this,  a 
dilatation  of  the  Haversian  canals 
and  a  poorness  in  lime  salts  of 
the  bony  tissue  is  found  histo- 
logically. 

Still  more  interesting  are  the 
"tabetic  arthropathies"  first  studied 
by  Charcot.  In  the  pathogenesis  of 
these  remarkable  joint  conditions, 
different  factors  combine.  Small 
fractures  in  the  region  of  the  ends 
of  the  bones,  and  particularly  'tear- 
ing off  of  tendons,  can  act  as  a 
starting-point,  or  the  hypotonic 
anomalies  of  position,  for  example,  the  genu  recurvation,  lead  to  stretch- 
ing of  the  capsular  apparatus  and  to  abnormal  friction  in  the  joint;  to  this 
is  added,  that  in  consequence  of  "deep  anesthesia,"  the  patient  in  such  cases 
does  not  in  the  least  spare  his  joint,  but  rather  maltreats  it  freely;  further, 
abnormal  secretory  conditions  in  the  synovia,  wearing  away  of  the  cartilage 
and  the  bone,  or,  on  the  contrary,  pathological  proliferation  in  them,  etc., 
come  into  play.  In  this  manner  exceedingly  striking  disease  pictures  occur, 
which  affect  far  most  frequently  the  knee,  then  in  diminishing  frequency  the 
ankle,  hip,  and  shoulder  joints.  The  greatly  distended  globular  joint  region, 
covered  with  tortuous  veins,  can  justify  the  comparison  with  the  abdomen 
of  a  dropsical  child  used  by  Brissaud.  At  other  times  marked  flail  joints, 
due  to  the  relaxation  of  the  capsular  and  ligamentous  apparatus,  occur;  or 
thi'  epiphysis  is  destroyed,  in  consequence  of  which  the  condyles  of  the  femur. 


Fig.  fi3. 
Tabetic  Foot. 


SYPHILOGEXIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     179 

the  head  of  the  femur,  or  of  the  humerus,  disappear,  and  the  shaft  of  the 
diaphysis  ends  free  in  the  empty  capsule  of  the  joint.  Besides  these  atrophic 
arthropathies,  hypertrophic  forms  occur,  in  which  the  proliferative  processes 
gain  the  upper  hand  and  portions  of  new-formed  bone  grow  into  the  joint 
cavities  and  throughout  the  periarticular  tissues  leading  to  rapid  ankylosis. 
Through  osteoarthropathies  in  the  region  of  the  root  and  middle  bones  of  the 
feet,  a  particular  kind  of  flat  foot,  the  so-called  "pied  tabetique"  (tabetic  foot) 
occurs  (Fig.  63). 

The  most  important  trophic  disturbance  of  the  integument  is  perforating 
ulcer  ("mal  perforant"),  which  in  the  great  majority  of  cases  is  located  on 
the  sole  of  the  foot  (particularly  on  the  ball  of  the  great  or  of  the  little  toe, 
or  on  the  heel).  This  is  a  round,  painless  ulcer,  which,  first  superficial,  grad- 
ually spreads  deeper  and  deeper,  and  finally  may  expose  a  joint  of  the  foot. 
As  "mal  perforant  palatin,"  Letulle  has  described  an  analogous  lesion  of  the 
palate  which  can  lead  to  perforation  of  this  through  into  the  nasal  cavity. 
While  these  ulcerations  are  usually  observed  in  the  early  stages  of  tabes,  in 
the  late  stages  there  is  a  tendency  to  extended  malignant  bed-sores,  which  are 
located  chiefly  over  the  sacrum,  but  also  in  other  places  (heels,  trochanters, 
elbows,  etc.).  A  rare  form  of  tabes,  the  "marantic  tabes"  of  Oppenheim,  is 
characterized  by  rapid  and  complete  disappearance  of  the  subcutaneous  fatty 
tissue.  As  farther  trophic  skin  symptoms  of  little  practical  importance,  erup- 
tions of  herpes,  hemorrhages  into  the  skin,  vitiligo,  ichthytosis  and  circum- 
scribed loss  of  hair,  are  observed  occasionally  in  tabetics.  Of  the  other  epi- 
dermal structures,  the  nails  (deformity,  brittleness,  falling  off)  and  the  teeth 
(painless  and  spontaneous  separation)   are  sometimes  affected. 

6.    RADICULAR   IRRITATIVE   PHENOMENA 

The  five  previously  considered  categories  of  posterior  root  symptoms — 
ataxia,  hypertonia,  arerlexia,  sensory  disturbances  and  trophic  disturbances — 
are,  as  we  have-  seen,  to  be  interpreted  as  due  to  impairment  of  function. 
We  must  now  pass  on  to  other  radicular  phenomena  which  plainly  present 
the  character  of  irritative  symptoms.  These  are  tabetic  hyperesthesias,  pares- 
thesias, pains  and  crises. 

Already  in  the  early  stages  of  the  disease  many  tabetics  complain  of  prick- 
ling, formication,  burning,  or  cold  feeling,  of  various  distribution,  now  located 
in  I  lie  trunk,  again  in  the  extremities.  On  the  trunk  the  circular  distribution 
of  these  paresthesias  sometimes  bring  it  about,  that  the  patients  sutler  con- 
tinuously from  a  feeling  of  constriction,  as  if  they  were  wearing  a  belt  or  a 
tightly  [accd  corset  around  thi'  body.  Hyperesthesias  also  occur  in  spots 
or  iii  girdle  form,  so  that,  for  example,  the  rubbing  of  the  shirt,  the  pressure 
of  the  shoe,  etc.,  may  become  almost  unbearable.  Particularly  frequent  is 
a    /one   of   marked    hyperesthesia    for   cold,  just    below    the    ribs. 

All  these  paresthesias  and  hyperesthesias  are  usually  quite  constant,  and 
last  through  long  periods  of  the  disease.  It  is  otherwise  usually  with  tabetic 
Bains,  which  almost  always  have  a  much  more  paroxysmal  character  than 
the  attacks   of  neuralgic   pain;  we   speak  hence  of  lightning  or   shooting  pains, 


180  LECTURE    XI 

"douleurs  fulgurantes  et  lancinantes."  Their  intensity  is  usually  very  great, 
sometimes  terrible,  so  that  the  patients  at  every  "stroke"  or  "stab"  writhe 
and  scream  aloud.  On  the  trunk  the  radicular  topography  of  the  radiations 
is  plainest  ("girdle  pain")  ;  in  the  extremities  less  so.  So,  many  patients 
express  themselves  as  having  the  feeling  as  if  a  knife  or  a  red-hot  iron  was 
suddenly  thrust  through  their  extremities  (by  far  most  frequently  into  the 
legs)  ;  others  have  the  sensation  of  being  struck  on  the  shin  with  a  club; 
still  others  of  the  flesh  being  torn  from  their  bones,  etc.  On  the  foot  the 
type  of  pressure  pain  is  particularly  frequent.  On  this  account  the  French, 
with  a  play  upon  the  "Spanish  boots"  of  the  torture  chamber,  speak  of  "dou- 
leurs en  brodequin."  Only  rarely  are  the  pains  more  continuous,  and  of  com- 
paratively steady,  often  very  moderate,  intensity,  so  that  such  patients  are 
for  a  long  time  considered  as  "rheumatics." 

Radicular  irritative  phenomena  in  the  region  of  the  internal  organs  we 
call  "crises."  They  are  always  accompanied  with  motor  or  secretory  phe- 
nomena reflexly  produced.  Gastric  crises  are  by  far  the  most  frequent.  At 
the  start  they  commence  with  severe  pain  in  the  abdomen  and  in  the  back,  and 
intense  retching  and  vomiting.  In  the  X-ray  picture  after  a  bismuth  or  barium 
meal,  the  spasmodic  contraction  of  the  stomach  to  hour-glass  form  has  been 
observed.  Chemical  examination  of  the  gastric  juice  usually  shows  marked 
hyperchlorhydria ;  however,  this  sometimes  is  absent;  indeed,  there  is  occa- 
sionally subacidity  in  gastric  crises.  These  excessively  painful  attacks,  which 
occur  with  great  disturbance  of  the  general  condition,  can  last  hours,  or  even 
days.  In  particularly  stubborn  cases  we  speak  of  a  "status  criticus,"  after 
the  analogy  of  the  "status  epilepticus."  In  them,  also,  in  consequence  of 
parenchymatous  ecchymoses  of  the  mucous  membrane  of  the  stomach,  there 
can  be  vomiting  of  blood  ("crises  noires").  The  cessation  of  gastric  crises  is 
just  as  abrupt  as  their  beginning,  and  even  greatly  reduced  patients  usually 
recover  quite  promptly  from  them. 

Other  crises  observed  in  tabes  are : 

Larynx  crises,  in  which  the  patient  is  suddenly  attacked  by  a  feeling  of 
suffocation  and  cough,  the  air  is  drawn  into  the  spasmodically  narrowed  glottis, 
with  difficulty  and  with  a  whistling  sound,  the  face  is  cyanotic,  and  the  patient 
breaks  out  in  a  sweat.  In  severe  cases  there  may  be  syncope ;  the  patient 
even  may  die ;  in  rudimentary  cases  the  attack  is  usually  limited  to  a  moderate 
"suffocating  cough."  As  in  all  other  crises,  the  return  to  normal  is  almost 
immediate. 

Pharynx  crises  consist  in  painful  swallowing  spasms,  oesophagus  crises  in 
painful  spasms  of  the  gullet,  heart  crises  resemble  angina  pectoris.  Before 
these  last  are  diagnosed  it  is  naturally  necessary  to  exclude  the  (quite  fre- 
quent)  coincidence  of  tabes  with  luetic  heart  and  vascular  disease. 

Sympathetic  crises  can  produce  fleeting  pictures  which  resemble  those  of 
Basedow's  disease  (see  Lecture  XXIII).  Vagus  crises  lead  to  paroxysmal 
alterations  of  the  heart  beat  and  of  the  breathing;  indeed,  to  apncea  and  syn- 
cope. I  have  interpreted  as  medullary  crises  attacks  of  hypcrpyretic  tem- 
perature with  Cheyne-Stohes  breathing. 

Irritative  phenomena  on  the  part  of  the  olfactory  nerve  with   spasms  of 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     181 

sneezing  and  rhinorrhcea,  show  nasal  crises,  colicky  pains  with  profuse  diar- 
rhoea, intestinal  crises.  The  rectal  and  vesical  crises  are  characterized  by  very 
severe  tenesmus.  We  have  already  alluded  to  testicular  crises  in  speaking  of 
the  differential  diagnosis  of  neuralgia  of  the  testicles  (see  page  53).  Renal 
crises  imitate  renal  colic,  liver  crises  that  due  to  gallstones.  The  clitoris  crises 
of  female  tabetics  are  characterized  by  spontaneous  paroxysms  of  sensuous 
feeling,  which  gradually  pass  over  into  painful  sensations ;  also  by  hyper- 
secretion of  the  vaginal  mucus  and  emptying  of  the  glands  of  Bartholin. 
Painful  attacks  of  singultus,  finally,  are  considered  as  diaphragmatic  crises. 


LECTURE    XII 

The  Syphilogenic  Diseases  of  the  Central  Nervous 

System 

A.     Tabes  Dorsalis  (Continued) 

Gentlemen  :  In  the  last  lecture  we  became  acquainted  with  those  com- 
ponents of  the  tabes  symptomatology  which  may  be  classed  together  as  "the 
posterior  root  symptom-complex"  and  for  whose  physio-pathological  explana- 
tion we  can  point  to  the  pathologico-anatomical  considerations  with  which  we 
preceded  our  remarks.  You  have  noted  in  the  description  of  the  "crises,"  that 
tabes  dorsalis  passes  beyond  the  bounds  of  the  spinal  innervation,  and  now  the 
description  of  its  important  ocular  symptoms  will  bring  still  more  clearly  before 
you  that  it  is  not  strictly  to  be  included  among  diseases  of  the  spinal  cord, 
but  is  rather  to  be  considered  as  a  cerebro-spinal  affection.  Fortunately,  our 
clinical  knowledge  of  this  subject  is  more  satisfactory  than  what  we  know  of 
its  pathologico-anatomical  and  physiological  basis,  hence,  since  so  much  in- 
formation is  still  lacking,  especially  as  to  the  explanation  of  the  pupillary 
symptoms,  I  prefer  not  to  enter  into  the  matter  in  order  to  avoid  becoming 
involved  in  a  discussion  of  theoretical  and  disputed  questions. 

OCULAR    SYMPTOMS 

1.  The  Pupillary  Symptoms. — Since  the  anomalies  presented  on  the  part 
of  the  pupillary  innervation  are  common  to  tabes  and  progressive  paralysis, 
our  description  of  these  will  be  applicable  to  what  may  be  found  in  all  the 
metasyphilitic  diseases  of  the  nervous  system.  In  a  great  many  cases  of  lues 
cerebro-spinalis  we  meet  with  the  same  conditions,  and  it  is  above  everything 
a  question  if  we  should  not  consider  them  as  pathognomonic  of  the  syphilogenic 
nature  of  an  existing  nervous  affection.  Personally,  I  am,  in  common  with 
many  other  neurologists,  of  this  last  opinion ;  indeed,  I  have  never  found  the 
most  important  of  the  clinical  manifestations  now  to  be  described,  Robertson's 
symptom,  in  typical  development  in  a  patient,  in  whom  a  non-syphilogenic  dis- 
ease had  to  be  diagnosed  as  was  shown  on  autopsy.  It  is  hence  just  as  much 
a  criterion  of  previous  syphilis  as  a  positive   Wasserinann  reaction. 

Testing  the  Pupillary  Functions. — Normally,  contraction  of  the  pupils 
("myosis,"  action  of  the  sphincter  pupilla'  innervated  from  the  oculo-motorius) 
occurs  under  the  following  conditions:  1.  By  the  action  of  light  upon  the  eye 
being  examined  or  upon  the  other  eye,  "direct  and  consensual  light  reaction." 
2.  As  a  regular  accompaniment  of  movements  of  the  other  eye  muscles,  among 

182 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     183 

which,  clinically,  only  movements  of  accommodation  and  convergence  come  into 
consideration  ( "synergic  pupillary  reaction").  3.  In  certain  individuals  upon 
the  basis  of  the  psychical  conception  "bright"  ("ideomotor  pupillary  re- 
action"). A  pupillary  dilatation  ("mydriasis"  through  the  dilator  pupilla' 
innervated  from  the  sympathetic),  on  the  other  hand,  occurs  normally  in  many 
individuals  when  a  painful  stimulus  is  applied,  particularly  to  the  face  or  neck, 
further,  in  fright,  anxiety,  orgasm,  etc. 

The  specific  action  of  many  alkaloids,  some  dilating,  others  contracting 
the  pupils,  is  well  known.  Mydriatics:  among  others,  are  atropin,  cocain, 
scopolamin,  duboisin.      Myotics:  physostigmin,  morphine,  pilocarpin. 

In  every  patient,  determining  the  morphological  relations  of  the  pupils 
should  precede  testing  their  reaction,  whether  they  are  round,  irregular,  of 
medium  width,  large  or  small,  if  they  are  of  the  same  size  on  both  sides  or  if 
there  is  difference  between  them  (anisocoria),  finally,  if  they  are  centrally  or 
eccentrically  located.  We  must  also  consider  further  whether  they  have  been 
acted  upon  by  any  of  the  above-mentioned  drugs  and  whether  there  has  been, 
or  is.  any  local  disease  {e.g.,  iritis)  which  could  alter  the  shape  and  function 
of  the  pupils.  Next,  we  examine  the  direct  reaction  to  light,  either  to  day- 
light (before  a  window)  or  to  artificial  light  (electric  lamp,  candle,  match,  etc.). 
The  patient  should  not  be  frightened,  however,  by  suddenly  throwing  a  strong 
light  into  his  eyes,  since  the  psychical  mydriasis  due  to  fright  may  inhibit  the 
light  myosis  and  cause  an  imperfect  reaction.  The  patient  must  further  look 
into  the  distance  in  this  test,  in  order  to  eliminate  synergic  convergence  and 
accommodation  reactions.  Further,  the  eye  not  being  tested  must  be  covered 
to  prevent  consensual  narrowing.  On  the  other  hand  we  test  the  last-men- 
tioned reaction  by  observing  the  iris  of  the  shaded  eye.  Usually  on  sudden 
illumination,  the  light  reaction  after  an  appreciable  period  of  latence  appears 
very  plainly.  Following  the  initial  contraction,  after  several  oscillations 
(physiological  hippus)  the  pupil  assumes  a  medium  width.  We  test  reaction 
for  a  convergence  and  accommodation,  by  having  the  patient  fix  his  eyes  on 
an  object  about  1  '  ^  meters  in  front  of  him,  and  then  approaching  it  slowly 
toward  his  nose. 

The  isolated  reflex  pupillary  rigidity,  or  the  Argyll-Robertson  symptom  of 
tabes  and  general  paresis,  manifests  itself,  in  that  the  pupil  dots  not  contract 
igither  when  exposed  directly  to  light,  or  when  the  other  eye  is  illuminated,  but 
does  react  in  accommodation  and  convergence.  The  Argyll-Robertson  symp- 
tom can  occur  unilaterally;  if  it  is  only  partial,  a  slight  direct  and  consensual 
light  reaction  can  still  be  obtained  on  strong  illumination,  and  we  speak  then  of 
reflex  sluggishness  of  the  pupils.  Reflex  rigidity  is  often  accompanied  by 
inyosis  and  irregularity  of  the  pupil,*  while  abnormal  width  of  one  or  both 
pupils  is  not  rare.  In  a  considerable  portion  of  cases,  anisocoria  is  present  at 
tome  stage  of  the  disease.  Where  this  is  variable,  so  that  sometimes  the  right 
fend    sometimes   the   left    eye   presents   a    wide   pupil,   we   speak   of  "bounding 

*  It  is  not  In  In-  forgotten  that  in  later  life,  and  especially  in  old  age,  the  pupils  arc  nor- 
mally narrower  than  in  young  people;  such  narrow  pupils,  however,  as  is  shown   by  man] 

tabetics  ("pinpoint"  pupils)  (In  nut  ciinic  within  the  physiological  limit.  Slow  reaction  to  light 
is  also  proper  to  old  age   (on  account  of  rigidity  of  the  iris). 


184  LECTURE    XII 

pupils."  Absolute  rigidity,  that  is,  inability  of  the  pupils  to  react  either  to 
light,  to  accommodation  or  to  convergence,  can  occur  in  tabes  and  paresis, 
but  here  it  is  to  be  considered  an  atypical  finding.  How  fundamentally  im- 
portant the  exact  examination  of  the  pupils  in  tabetics  is,  is  shown  by  the 
statistics  of  Faure  and  Dcsvaulx  who,  in  200  cases  found  pupillary  anomalies 
of  some  sort  193  times. 

2.  Tabetic  Diseases  of  the  Optic  Nerve. — Upon  the  basis  of  tabes  (as 
well  as  upon  that  of  progressive  paralysis  and  indeed,  as  an  independent  mono- 
svmptomatic,  metaluetic  affection  of  the  optic  nerve)  a  simple  noninflammatory 
atrophy  of  the  optic  nerve  can  develop.  There  is  a  primary  degeneration  of 
the  optic  neurones  with  reparatory  proliferation  of  the  supporting  tissue,  that 
is,  the  pathologico-anatomical  analogue  of  the  tabetic  degeneration  of  the 
posterior  roots  and  posterior  columns,  which  is  more  to  be  dreaded  in  the 
early  stages  than  in  advanced  tabes.  According  to  Uhthoff,  optic  atrophy 
occurs  in  10  per  cent,  to  15  per  cent,  of  tabetics;  according  to  my  personal 
experience  this  percentage  seems  to  me  too  high.  As  the  study  of  the  visual 
field  shows,  the  so-called  "papillomacular  bundle"  of  the  optic  nerve  remains 
longest  intact,  so  that  there  is  concentric  narrowing  of  the  visual  field;  less 
frequent,  are  irregular  losses  of  vision,  entirely  atypical,  central  scotoma. 
Color  perceptions  (particularly  for  green)  are  usually  lost  before  those  for 
white.  Ophthalmoscopically  the  general  pallor  of  the  nerve  head  is  next  ob- 
served; little  by  little  it  becomes  chalk-white,  on  which  account  its  outlines 
remain  very  sharp.  Finally,  the  vessels  are  also  affected  and  appear  much 
thinned.  Where,  on  the  other  hand,  the  atrophy  develops  from  a  neuritic  process 
(that  is,  the  papilla  appears  cloudy,  washed  out)  there  is  a  complicating 
syphilitic  disease  of  the  optic  nerve  and  not  a  metaluetic,  purely  degenerative 
atrophy.  This  last  is  therapeutically  little  to  be  influenced  and  of  very  bad 
prognosis;  it  leads  in  a  short  time  (1  to  3  years)  to  complete  blindness.  Only 
very  exceptionally  does  the  degenerative  process  come  to  a  standstill. 

3.  Paralyses  of  the  Eye  Muscles. — In  contradistinction  to  most  of  the 
tabes  symptoms  previously  considered,  which  are  characterized  by  their  ten- 
dency to  progress,  or  at  least  to  remain  constant,  the  tabetic  eye-muscle  paraly- 
ses are,  in  the  majority  of  cases,  of  temporary,  even  fleeting  nature.  These  phe- 
nomena, also,  usually  belong  to  the  early  stage  of  tabes,  in  which  the  paralysis 
affects  b}r  preference  a  single  muscle  ("paralysies  parcellaire"  of  Fournier), 
most  frequently  the  external  rectus,  but  little  more  rarely  the  internal  rectus 
or  the  levator  palpebral.  Where,  on  the  other  hand,  the  eye  muscle  paralyses 
appear  in  the  late  stages  of  the  disease,  they  are  usually  of  greater  extent. 
Strabismus,  double  vision,  ptosis,  are  their  clinical  results.  We  are  not  entirely 
clear  either  as  to  the  location  or  as  to  the  nature  of  the  pathological  substratum 
of  these  paralyses,  whether  there  are  changes  in  the  nuclei  or  in  the  nerve  trunks. 
The  circumstance  that  these  disturbances  often  last  only  a  few  days,  further, 
can  vary  from  one  day  to  another,  seems  to  justify  the  suspicion  that  they  are 
due  to  circulatory  disturbances,  probably  to  vascular  spasm.  Permanent  or 
even  progressive  eye  muscle  paralyses  in  tabetics  we  consider  rather  as  tertiary 
luetic  complications. 


SYPHELOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     185 

OTHER    CRANIAL    NERVE    SYMPTOMS 

Along  with  the  so  important  ocular  phenomena  of  tabes  dorsalis,  its  mani- 
festations in  the  other  cranial  nerves  play  only  a  subordinate  role.  The  involve- 
ment of  the  auditory  nerve  we  have  not  rarely  observed  in  the  form  of  a  progres- 
sive nerve  deafness,  with  diminution  of  hearing  through  bone  conduction  (to  be 
demonstrated  by  the  Schwabach,  Rhine  or  Weber  tests — see  Lecture  II,  page 
34),  partial  loss  of  perception  for  the  musical  scale,  tinnitus  and  occasionally, 
also,  attacks  of  labyrinthine  vertigo.  Oppenhelm  and  Siemerling  among  others 
have  recognized,  anatomically,  the  degeneration  of  the  auditory  nerve.  Re- 
duction of  the  olfactory  and  gustatory  perceptions,  which  may  increase  to 
anosmia  and  ageusia,  and  which  Klippell  has  attributed  to  destruction  of  fibers 
in  the  olfactory  and  glosso-pharyngeal  nerves,  are  rare.  In  the  trigeminus, 
paresthesias,  hypesthesias  and  anesthesias  occur;  in  the  facial,  unilateral  or 
bilateral,  usually  temporary  paralyses  (exceedingly  rare).  In  the  hypoglossus, 
Raymond,  Cassirer-Schiff  and  others  have  observed,  as  an  excessive  rarity, 
hemiatrophy  of  the  tongue. 

Symptoms  of  disturbance  in  the  innervation  of  the  larynx  arouse  great  in- 
terest. They  are  far  more  frequent  than  the  already  mentioned  larynx  crisis 
with  which  they  are  classed  by  the  French  under  the  name  "laryngisme  tabet- 
ique."  According  to  Dcjerine,  paralyses  of  the  laryngeal  muscles,  or  ataxia  of 
the  vocal  cords,  occur  in  nearly  -i.5  per  cent,  of  tabetics,  the  former  affect 
by  preference  the  muscles  opening  the  glottis,  that  is,  the  posterior  cricoaryte- 
noids. Pareses  of  the  posterior  muscles,  of  slight  degree  can,  particularly  when 
they  are  unilateral,  cause  no  clinical  symptoms,  and  may  only  be  discovered 
on  laryngoscopy.  In  severe  cases  the  air  is  drawn  in  with  a  whistling  sound, 
and  slight  bodily  exertion  leads  to  severe  dyspnoea;  on  the  other  hand,  phonation 
is  uninfluenced.  More  rare,  are  disturbances  in  the  closers  of  the  glottis  which, 
when  they  are  present  bilaterally,  show  themselves  through  bitonality  of  the 
voice,  "breaking,"  etc.,  when  unilateral,  however,  remain  latent.  In  ataxia 
of  the  vocal  cords  the  voice  is  tremulous,  sensory  disturbances  of  the  larynx 
(hyperesthesia  or  anesthesia)  are  also  occasionally  observed.  All  these  mani- 
festations belong  to  no  particular  stage  of  the  disease,  but  occur  now  as  early 
again  as  late  symptoms.  They  may  be  inconstant,  and  in  the  same  patient  are 
sometimes  present,  sometimes  absent. 

DISTURBANCES    IN    THE    SKELETAL    MUSCLES 

In  general  the  muscles  of  the  tabetic  remain  normal  until  the  disease  is  very 
far  advanced  and  has  led  to  cachexia  and  great  loss  of  strength,  then  we  almost 
always  see  a  wasting  of  the  musvles  without  fibrillary  contractions  or  reaction 
of  degeneration,  in  which  the  feet  of  the  permanently  bedridden  patient,  partly 
on  account  of  the  hypotonia  of  the  peroneal  muscles  which  are  no  longer  able 
to  hold   up  the  weight   of  the  feet,  partly  from  pressure  of  the  bedclothes,  etc., 

may  take  an  equinovarus  position.   Sometimes,  however,  circumscribed  muscular 

atrophies   occur,  even   in   the  early   stages   of  the  disease,  usually   in    the   upper 
extremities    (shoulder-girdle,    small    muscles    of    the   hand),    in    which    sometimes 


186  LECTURE    XII 

partial  reaction  of  degeneration,  sometimes  only  quantitative  reduction  of 
irritability,  is  to  be  found.  Corresponding  to  tbe  degree  of  atrophy,  muscular 
power  is  naturally  reduced,  so  that  finally  marked  parescs  or  even  paralyses 
may  result.  These  atrophic  phenomena  are,  as  far.  as  their  pathogenesis  is  con- 
cerned, scarcely  to  be  considered  as  anything  unique.  In  many  cases  they  may 
present  the  analogue  of  the  already  mentioned  trophic  disturbances  of  the 
skeleton,  integument,  etc. ;  in  others,  however,  which  have  been  distinguished  as 
"amyotrophic  tabes,"  there  may  be  a  combination  of  tabes  dorsalis  with  "syphi- 
litic spinal  amyotrophy"  (Raymond,  Lcri,  Nonne,  etc.),  which  depends  upon 
disease  of  the  anterior  horns  of  the  spinal  cord. 

GENITAL  AND  SPHINCTER  DISTURBANCES 

One  of  the  first  symptoms  of  tabes  in  men  is  usually  impotence ;  there  are, 
however, cases  in  which  potence  is  very  long  preserved  ;  usually  a  period  of  sexual 
excitement  precedes  the  development  of  sexual  weakness ;  this  may  be  responsible 
for  the  erroneous  view  of  the  older  authors  and  which  is  still  in  existence  among 
the  laity,  that  sexual  excesses  may  have  tabes  as  a  result.  Anesthesia  of  the 
glans  is  often  to  be  found  along  with  disappearance  of  the  power  of  erection. 
The  genital  disturbances  of  female  tabetics  present  themselves  apart  from  the 
clitoris  crises  mentioned,  above  everything  in  anaphrodisia  or  indeed,  as  total 
anesthesia  of  the  genital  organs ;  labor  sometimes  runs  its  course  entirely  without 
pain  and  may  be  accompanied  by  insufficient  expulsive  power  and  atonic  hemor- 
rhages. 

The  bladder  function  very  frequently  suffers  impairment  and,  indeed,  in 
various  forms.  Sometimes  the  patient,  in  spite  of  great  desire  to  urinate,  must 
strain  a  long  time  before  he  can  pass  water;  sometimes  the  desire  is  immediately 
followed  by  emptying  the  bladder  and  the  patient  wets  himself,  or,  on  the  other 
hand,  the  feeling  of  desire  to  urinate  is  lost  entirely  and  the  patient  only  urinates 
"out  of  consideration,"  as  Fournier  expresses  it.  All  these  disturbances  are 
often  already  present  in  the  early  stages  of  tabes,  but  are  in  no  way  constant ; 
rather  are  frequent  intermissions,  with  entire  return  to  the  normal,  as  well  as 
great  variations  in  the  intensity  of  the  anomalies,  quite  frequently  observed. 
Even  severe  symptoms,  as,  for  example,  complete  retention  which  necessitates 
catheterism,  can  completely  disappear  after  lasting  for  months.  There  occurs, 
further,  ischuria  paradoxa,  in  which  discharge  of  urine  (in  drops)  only  occurs 
when  filling  of  the  bladder  has  reached  a  certain  degree,  incomplete  retention,  in 
which  the  bladder  can  never  be  completely  emptied  and  a  certain  quantity  of 
residual  urine  remains  in  it ;  finally,  true  incontinence.  This  last,  characterized 
by  continual  dribbling  of  urine  and  a  consequence  of  atony  of  the  sphincter  and 
of  the  detrusor  vesica?,  belongs  to  the  late  stages  of  tabes.  It  may  be  only 
nocturnal,  or  can  manifest  itself  also  in  the  waking  state.  Only  exceptionally, 
and  then  only  in  the  terminal  stages,  incontinence  of  the  bowels  occurs  in 
tabetics,  while  obstinate  constipation,  on  account  of  intestinal  atony,  is  quite 
frequent. 


SYPHILOGEXIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     187 

THE    CONDITION    OF    THE    CEREBROSPINAL    FLUID 

When  I  now  pass  over  to  the  consideration  of  this,  of  late  so  freely  discussed 
questions  of  the  anomalies  in  the  cerebro-spinal  fluid  in  tabes  and  their  deter- 
mination— we  will  consider  here  also  paresis  and  cerebro-spinal  syphilis — I 
would  warn  you  against  overestimation  of  results  obtained  by  these  methods 
and  the  too  frequent  making  of  lumbar  punctures  in  all  patients  with  syphi- 
litic nervous  diseases.  While  this  procedure,  carried  out  with  proper  technique, 
is  scarcely  dangerous  (see  Lecture  XVI),  it  is  best  reserved  for  a  specialist 
who  is  also  skilled  in  the  necessary  laboratory  investigations.  He,  also,  should 
only  use  it  where  an  extended  clinical  investigation  still  leaves  the  diagnosis 
in  doubt. 

Within  the  limits  of  these  lectures  I  cannot  go  into  the  technique  of  the 
Wassermann  complement-fixation  reaction  which  is  to  be  applied  in  the  first 
place  to  any  spinal  fluid  removed  on  account  of  suspicion  of  a  syphilogenic 
affection.  (The  amount  of  fluid  necessary  for  making  all  the  necessary  investi- 
gations is  about  5  or  6  cc.)  The  second  test  to  be  applied  to  the  fluid  is  the 
globulin  reaction  of  Xonne-Apelt.  To  the  fluid,  equal  parts  of  a  hot  saturated 
and  then  cooled  solution  of  ammonium  sulphate  is  added;  if  in  three  minutes 
( "Phase  I"  )  there  is  clouding,  this  speaks  for  abnormal  globulin  or  nucleoal- 
bumin  content  of  the  specimen  under  examination.  The  third  test  consists 
in  the  cvtological  examination  of  the  centrifugated  sediment  (Widal,  Ravaut, 
ffissl )  :  while  normally  only  occasional  lymphocytes  are  found,  marked  lympho- 
cytosis is  to  be  considered  as  a  certainly  pathological  finding.* 

The  indications  of  these  reactions  to  which,  as  a  fourth,  the  Wassermann 
test  applied  to  the  blood  serum  is  to  be  added,  in  the  diagnosis  of  syphilitic 
diseases  of  the  central  nervous  system,  is  best  shown  in  the  following  synopsis 
of  Nonne: 

I.     Blood   Examination 
Wassermann  Reaction. 

a.  Positive. — Is  characteristic  for  syphilis,  with  a  few  exceptions  coming 
little  or  not  at  all  into  practical  consideration.  A  positive  reaction  is  given 
also  by  a  few  cases  of  scarlatina  (only  at  certain  very  limited  stages  of  the 
di-ease),  by  malaria,  by  framboesia,  by  lepra,  by  the  plague,  etc. 

A  positive  Wassermann  reaction  from  the  blood  scrum  tells  nothing  further 
than  that  the  individual  under  consideration  has  at  some  time  had  syphilis, 
hereditary  or  acquired,  not  that  the  disease  now  present  must  be  of  luetic  nature. 

I).  Negative. — Is  to  be  considered  as  speaking  against  paresis  since  with 
exceedingly  rare  exceptions  the  blood  of  parities  gives  a  positive  Wassermann 
reaction. 

II.     Examination   ok   THE  Spinal   Fi.ru> 

a.  Normal  Fluid.—  Pressure  !'()  to  180  mm  water  (Manometer).  Phase  1 
reaction  negative;  at  most  5  to  0  cells  to  the  cu  mm  (Fnehs-Rosenthal  counting 
Apparatus). 

"  By  many  laboratory  workers,  in  the  United  States  at  any  rate,  the  Noguchi  butyric  acid 
test  for  globulin  is  considered  more  delicate  than  thai  of  Nonne-Apelt.    Also  it  is  customary  to 

eounl    the   cells   in    (lie    fluid    willum!    centrifugating   it.—  Translator. 


188  LECTURE    XII 

Wassermann  reaction,  applied  according  to  the  original  method,  using  0.2 
cc  of  the  fluid  to  be  examined  and  also  with  larger  quantities  (0.3-1.0  cc 
fluid)  negative. 

b.  Pathological  Fluid. — 

1.  Discharged  under   increased   pressure    (over    150   mm   water). 

2.  Positive  phase,  1  reaction. 

3.  Increased  cell  content. 

These  three  symptoms,  in  combination  or  alone,  show  that  there  is  an 
organic  disease  of  the  central  nervous  system  (specified  or  non-specific). 

4.  Whether  the  disease  of  the  central  nervous  system  is  of  syphilitic  nature 
is  decided  by  the  Wassermann  reaction  applied  to  the  spinal  fluid. 

If  the  Wassermann  reaction,  applied  according  to  the  original  method  (0.2 
cc  fluid  being  used),  is  positive  there  is  the  greatest  probability  that  the  case 
under  investigation  is  paresis  or  tabo-paralysis,  much  more  rarely  is  it  cerebro- 
spinal syphilis,  and  only  in  exceptional  cases  true  tabes. 

In  the  great  majority  of  cases  of  paresis  the  Wassermann  reaction  is  posi- 
tive with  0.2  cc  of  spinal  fluid. 

In  a  few  cases  of  paresis,  in  almost  all  cases  of  cerebro-spinal  syphilis  and 
tabes,  the  Wassermann  reaction  is  only  positive  when  larger  quantities  of  fluid 
(0.3,  0.4  to  1.0  cc)  are  used. 

TYPICAL    FINDINGS 
/.  Paresis  or  Taboparalysis. 

1.  Wassermann  reaction  in  the  blood  positive  (in  almost  100  per  cent,  of 
cases).  Lumbar  pressure  frequently  increased. 

2.  Phase  1,  reaction  positive  (in  about  95  to  100  per  cent,  of  cases). 

3.  Lymphocytosis  (in  about  95  per  cent,  of  cases). 

Wassermann  on  the  spinal  fluid: 

a.  Positive  in  about  85  to  90  per  cent,  when  the  original  method  is  used; 
(0.2  cc  fluid). 

b.  Positive  in  100  per  cent,  when  larger  quantities  of  fluid  are  used. 

//.   Tabes  without  Combination  with  Paresis. 

1.  Wassermann  reaction  in  the  blood  serum,  positive  in  60  to  70  per  cent, 
of  cases ;  lumbar  pressure  frequently  increased. 

2.  Phase  1,  reaction  positive  in  about  90  to  95  per  cent,  of  cases. 

3.  Lymphocytosis   positive   in   about   90   per   cent,    of   cases. 

4.  Wassermann  on  the  spinal  fluid: 

a.  Original  method  (0.2  cc),  positive  in  5  to  10  per  cent. 

b.  Larger  amounts  of  fluid,  positive  in  almost  100  per  cent. 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     189 

III.  Cerebrospinal  Syphilis. 

1.  Wassermann  reaction  in  blood  serum  positive  in  about  80  to  90  per  cent. ; 
lumbar  pressure  frequently  increased. 

2.  Phase  1,  reaction  negative  only  in  exceptional  cases,  otherwise  positive. 

3.  Lymphocytosis  like  Phase  1,  almost  always  positive. 

4.  Wassermann  on  the  spinal  fluid: 

a.  Original  method  (0.2  cc)  positive  in  about  10  per  cent. 

b.  With  larger  quantities  of  fluid,  almost  always  positive  (particularly 
valuable  in  differential  diagnosis  from  multiple  sclerosis,  brain  tumor  and  spinal 
cord  tumor). 

Course  and  Prognosis 

The  average  duration  of  tabes  can  be  placed  at  from  10  to  20  years.  On 
the  one  hand,  however,  malignant  cases  which  lead  to  death  in  a  few  years 
occur,  and  on  the  other  there  are  those  which  characterized  by  special  mild- 
ness, slow  progression,  complete  standstill,  or  even  partial  regression  of  the 
symptoms,  permit  reaching  an  advanced  age. 

For  the  prognostic  estimation  of  the  individual  case  the  following  prin- 
ciples are  applicable  in  general,  though,  like  all  rules,  they  of  course  have  their 
exceptions :  Mildness  of  course  is  the  more  to  be  expected,  the  later  after  luetic 
infection  the  disease  becomes  apparent,  the  longer  also  the  latent  or  incubation 
stage  of  tabes  has  lasted ;  with  this  well  agrees  the  fact  that  cases  of  tabes  in 
the  mature  period  (beginning  about  the  5th  decade)  are  usually  characterized 
by  their  mildness.  A  tendency  to  attacks  of  pain  persisting  for  a  long  time,  al- 
though this  peculiarity  greatly  influences  the  comfort  of  the  patient,  is  usually 
associated  with  favorable  prognosis  as  regards  preservation  of  ability  to  walk 
and  duration  of  life.  On  the  other  hand  the  richness  in  symptoms  of  a  con- 
crete case  of  tabes,  is  to  be  considered  unfavorable  prognosticallv  witli  the 
single  qualification  that  complication  with  paresis  in  typical  forms  of  tabes  with 
many  symptoms,  is  scarcely  to  be  feared.  The  same  remark  applies  in  greater 
measure  to  Hie  cases  of  tabes  characterized  by  location  of  the  lesion  high  up 
(Tabes  superior)  in  which  variety  the  prognosis  is  clouded  by  fear  of  complica- 
tion witli  bulbar  symptoms.  The  estimation  of  the  general  condition  is  very  im- 
portant :  Syphilogenic  heart  and  vascular  lesions  aii'  not  at  all  rarely  found 
in  tabetics  on  close  investigation  and  where  present  alter,  markedly  tor  the 
Worse,  the  outlook.  Finally,  the  striking  fact  is  to  be  considered  that  the 
Occurrence  id'  blindness  from  tabetic  optic  atrophy  ("amaurotic  tabes")  very 

Often  puts  a  stop  to  the  further  development  of  the  disease,  so  that  among 
l'ri  neh  authors  the  expression  ''Tabes  arrested  by  blindness"  is  in  common  use. 
In  a  somewhat  schematic  but  practically  quite  useful  manner,  three  stages 
in  I  lie  development  of  tabes  are  distinguished  from  one  another;  the  preatactic, 
ill  which  tin'  gait  is  not  yet  altered;  the  atactic,  in  which  it  is  characteristically 
altered  on  account  of  the  disturbances  of  coordination;  and  the  paralytic,  in 
which  a  paralysis  is  combined  with  advanced  ataxia,  so  that  the  patient  is  per- 
manently bedridden.  Tabes  is  the  cause  of  death  only  in  very  rare  cases  (par- 
alysis of  the  larynx,  paralysis   of  Hie  bladder,  cystitis   and   uremia;   bed-sores. 


190  LECTURE    XII 

and  sepsis;  inanition  and  exhaustion  after  protracted  gastric  crises;  status 
criticus,  vagus  crises,  etc.)  ;  death  usually  occurs  from  intercurrent  diseases 
(tuberculosis,  pneumonia,  influenza,  etc.),  against  which  the  power  of  resist- 
ance is  greatly  reduced,  not  at  all  infrequently  from  syphilogenic  diseases  of 
the  vessels  (angina  pectoris,  aneurism  of  the  aorta,  cerebral  hemorrhage,  etc.). 
We  will  now  enumerate  the  courses  which  tabes  dorsalis  may  take.  Accord- 
ing to  its  rudimentary  mildness  or  malignancy  there  have  been  separated  on  the 
one  side  rudimentary  or  abortive  tabes,  in  which  the  patients  never  get  beyond 
the  preatactic  stage  (I  know  of  cases  which  have  been  in  existence  30  years)  ; 
on  the  other  hand,  tabes  acutissima,  "galloping  consumption  of  the  spinal  cord," 
which  in  one  of  my  cases  in  9  months  ran  through  the  atactic  and  paralytic 
stages  and  led  to  death  in  cachexia.  Through  the  time  of  its  beginning  the  very 
rare  juvenile  tabes  dorsalis  is  characterized,  commencing  in  youth  or  even  in 
late  childhood,  it  arises  upon  a  hereditary  luetic  basis.  According  to  its  loca- 
tion, special  positions  are  taken  by  tabes  superior  or  cervical  tabes,  in  which  the 
anomalies  att'ect  the  arms,  and  the  lower  limbs  present  loss  of  patellar  reflex 
as  their  sole  tabetic  symptom ;  and  the  opposite  condition,  tabes  of  the  conus 
terminalis.  A  case  of  this  last  variety  affecting  a  43-year-old  woman,  care- 
fully investigated  by  Thomas  and  myself,  presented  the  following  symptoms: 
Rectal  crises,  incontinence  of  urine  and  stools,  total  anesthesia  of  the  genitals 
(so  that  coitus  was  not  at  all  noticed),  total  peri-ano-genital  anesthesia  (of  the 
riding  trouser  form),  sometimes  pain  in  the  legs,  myosis,  reflex  rigidity  of  the 
pupils,  exaggeration  of  the  patellar  reflexes.  A  special  topographic  variety  is 
also  tabes  amaurotica,  of  whose  frequent  inhibitory  influence  upon  the  further 
extension  of  the  spinal  lesions  we  have  already  spoken ;  simple  metasyphilitic 
optic  atrophy  can  be  considered  indeed,  as  a  "tabes  without  spinal  cord  symp- 
toms." Finally,  according  to  the  special  preponderance  of  individual  symptoms 
or  symptom-groups,  we  speak  of  a  tabes  dolorosa,  visceralis,  amyotrophia, 
marantica. 

Diagnosis  and  Differential  Diagnosis 

The  diagnosis  of  fully  developed  tabes  is  one  of  the  easiest  tasks  in  clinical 
neurology  and  can  often  be  made  at  a  glance.  In  the  early  stages  the  dis- 
turbances are  less  striking,  so  that,  unfortunately,  disastrous  mistakes  are 
frequent  enough;  so,  for  example,  gastric  crises  have  been  mistaken  for  ulcer 
of  the  stomach  and  laparotomized,  also  patients  with  lancinating  pains  are 
continually  sent  into  the  hospitals  with  diagnoses  of  rheumatism,  neuralgia, 
sciatica,  etc.  Nevertheless,  even  in  the  early  stages,  tabes  can  be  recognized 
nearly  always  with  certainty,  after  a  thorough  general  examination.  In  tak- 
ing the  history  and  estimating  the  present  condition  the  following  "cardinal 
symptoms"  should  be  specially  sought  after: 

1.  Pupillary  disturbances  (reflex  rigidity  or  slowness,  anisocoria,  irregu- 
larity, myosis). 

2.  Localized  or  radicular  ( that  is  on  the  extremities  in  longitudinal 
bands,  on  the  trunk  as  zones)  hvpesthesias  or  hyperesthesias  (the  last  parti- 
cularly for  cold),  anesthesias  of  the  nerve  trunks  or  viscera. 


SYPHILOGENIC  DISEASES  OE  CENTRAL  NERVOUS  SYSTEM     191 

3.  Reduction  or  loss  of  tendon  reflexes  (their  exaggeration  m  no  way  ex- 
eludes  tabes  incipiens). 

4.  Romberg's  symptom,  sometimes  only  to  be  recognized  when  standing  on 
one  leg. 

5.  Lancinating  pains  or  crises. 

6.  Bladder  or  genital  disturbances. 

7.  Optic  atrophy. 

If  four  of  these  symptoms  are  found  the  diagnosis,  tabes  dorsalis,  is  abso- 
lutely assured,  if  three,  it  is  almost  certain.  A  probable  diagnosis  can  be  made 
from  two  of  these  symptoms  and  eventually  confirmed  by  the  results  of  blood 
and  spinal  fluid  examination.  The  laboratory  methods  are  a  diagnostic  neces- 
sity, only  in  the  quite  rare  cases  of  beginning  tabes,  where  most  exact  clinical 
examination  shows  only  one  cardinal  symptom. 

The  following  compilation  after  Oppenheim  may  give  you  a  conception 
of  the  manifoldness  of  the  symptom  grouping  in  tabes  incipiens: 

1.  Lancinating  pains,  WestphaVs  symptom,  pupillary  rigidity. 

2.  Bladder  weakness,  West phuVs  symptom,  girdle  symptom. 
.'J.      Pupillary  rigidity,  anesthesia  on  the  trunk. 

4.  Optic  atrophy,   WestphaVs  symptom,  analgesia. 

5.  Optic  atrophy,  girdle  symptom  with  corresponding  hypesthesia, 
analgesia. 

<i.  Optic   atrophy,   lancinating   pains,   impotence. 

7.  Attacks  of  vomiting,  WestphaVs  symptom. 

8.  Attacks  of  vomiting,  loss  of  the  Achilles'  tendon  reflex. 

9.  Attacks  of  vomiting,  pupillary  rigidity. 

10.  Gastric  crises,  anesthesia  on  the  trunk. 

11.  Joint  affection,  analgesia,  West plial's  symptom. 

12.  Joint  affection,  analgesia,  pupillary  rigidity. 

13.  Paralysis  of  the  vocal  cords  (with  or  without  attacks  of  coughing). 
WestphaVs  symptom,  pupillary  rigidity. 

14.  Spontaneous  falling  out  of  the  teeth,  disturbances  of  sensation  in  the 
trigeminus  distribution,   WestphaVs  symptom,  bladder  trouble. 

15.  Paralysis  of  eye  muscles,  girdle  symptom,  analgesia. 

16.  Optic  atrophy,  anesthesia  on  tin-  trunk. 

Of  gnat  differentia]  diagnostic  importance  is  the  distinction  of  tabes  dorsalis 
from  "peripheral  pseudo-tabes"  as  it  occurs  in  diphtheritic  and  alcoholic  poly- 
neuritis. (Sec  above,  page  42.)  The  Argyll-Robertson  symptom  occurs  in 
mural  tabes  almost  never  (that  is,  only  when  the  patient  is  an  old  syphilitic). 
Further  tabes  symptoms  foreign, to  polyneuritis,  are  gastric  crises  in  typical 
development  ( gas!  ric  disturbances,  nasi  ralgia,  I  he  morning  vomit  ing  of  drinkers, 
I  tc.  as  they  are  not  rare  in  patients  with  alcoholic  neuro  tabes,  must  not  be  mis- 
taken for  crises),  other  paroxysmal  visceral  disturbances,  viscera]  anesthesias. 
Bladder  disturbances  are  very  rare  in  polyneuritis  and  almost  never  reach  any 
Considerable  extent;  a  limitation  of  this  remark  applies  only  to  those  cases  in 
which  a  "polyneuritic  psychosis"  devi  lops  and  leads  to  incontinence  of  urine  and 


192  LECTURE    XII 

feces.  The  condition  of  the  peripheral  nerve  trunks  on  palpation  is  important : 
In  tabes  usually  hypo-  or  anesthetic  to  pressure,  they  are  never  so  in  neuritic 
pseudo-tabes,  but  are  usually  very  sensitive  to  pressure  and  besides  are  often 
thickened.  The  eventual  superficial  anesthesias  of  the  atactic  forms  of  poly- 
neuritis have  not  a  radicular  topography,  but  increase  from  the  roots  of  the 
extremities  toward  the  periphery  and  reach  their  maximum  on  the  ends  of  the 
limbs.  I  have  never  seen  girdle  sensation  and  anesthesia  in  girdle  form,  in  poly- 
neuritis. Lancinating  or  boring  pains,  preceding  by  years  the  onset  of  ataxia, 
occur  only  in  tabes.  The  early  appearance  of  degenerative  paralyses  or  pareses 
(namely  in  the  peroneal  region  with  ''steppage,"  see  above,  page  23),  speak 
against  tabes.  The  affections  of  the  optic  nerve  occurring  eventually  in  both 
diseases,  are  to  be  differentiated  without  difficulty  by  ophthalmological  exam- 
ination. With  the  ophthalmoscope,  in  retro-bulbar  neuritis  a  paling,  tempo- 
rally located,  is  found  instead  of  the  diffuse  pearly  or  porcelain  white  coloration 
in  tabes;  decisive  is  the  difference  in  the  limitation  of  the  visual  field;  in  poly- 
neuritis, central  scotoma,  in  tabes  almost  always  peripheral  narrowing.  As  to 
the  development  of  the  disease,  "acute  ataxia"  occurs  far  more  frequently  from 
"neurotabes  peripherica."  This  increases  rapidly  but  has  a  much  greater 
tendency  to  regression,  even  to  cure,  of  the  symptoms,  than  tabes  dorsalis. 

Treatment 

Although  it  is  of  course  not  permitted  to  us  to  cause  regression  of  the  de- 
generation of  important  nerve  elements,  which  is  the  basis  of  tabes,  and  although 
in  many  cases  the  progress  of  the  disease  is  inexorable,  nevertheless,  the  great 
majority  of  tabetics  give  us  plenty  of  opportunity  for  active  interference  in  the 
sense  of  symptomatic  therapy. 

Before,  however,  we  enter  upon  this  last,  we  must  take  some  stand  in  the 
much  disputed  question  as  to  how  far  a  causal  treatment  of  tabes  dorsalis  is  to 
be  attempted — and  who  speaks  here  of  causal  therapy  can  readily  speak  of 
"specific"  or  "anti-luetie"  treatment. 

It  is  not  only  certain  that  thorough  treatment  of  syphilis  does  not  exclude 
later  development  of  tabes  or  paresis,  but  it  is  indeed  questionable  if  it  to  any 
extent  reduces  the  risk  of  these  diseases.  This  we  can  affirm  even  without  the 
extensive  and  faultless  statistics  which  to  date  are  still  wanting.  Less  extensive 
statistics,  however,  as,  for  example,  those  of  Spillmann,  who  among  32  thor- 
oughly treated  syphilitics  later  saw  not  a  single  one  attacked  by  metaluetic 
disease  cannot  be  considered  conclusive.  Reumont  has  shown,  as  already  men- 
tioned, that  in  syphilitics  only  about  1  per  cent,  later  become  tabetic.  If  the  in- 
troduction of  the  salvarsan  treatment  alters  the  matter  we  must  wait  and  see. 
It  is  at  least  certain  that  among  our  tabetics  and  paretics,  such  patients  as 
have  at  the  time  of  their  syphilis  been  thoroughly  mercurialized  are  not  few  in 
number.*     That  these  cases,  indeed,  present  only  a  minority  is  not  remarkable, 

*  I  would  refer  to  four  double  observations  of  conjugal  parasyphilis,  in  which  the  husband 
on  his  part  went  through  a  specific  cure;  the  wife,  however,  remained  untreated,  and  both  devel- 
oped tabes  or  paresis  about  the  same  time,  or,  rather,  the  man  earliest.  See  above  (page  165) 
what  was  said  about  "Syphilis  a  virus  nerveux." 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     193 

since  thorough  treatment  is  received  by  only  very  few  luetics.  Also  the  para- 
doxical result  of  the  retrospective  statistics  of  Evlenburg,  Dinkier,  Kron, 
Schuster,  and  Mend  el -Tobias  cannot  be  disregarded;  just  the  patients  of  these 
authors  who  had  had  the  most  energetic  anti-syphilitic  treatment  showed  the 
shortest  incubation  period  of  their  tabes.  It  would  be,  of  course,  foolish  on  this 
account  to  assert  that  there  was  inadequate  treatment  of  the  syphilis,  but  such 
statistics,  however,  are  a  warning  against  too  schematic  handling  of  the  ques- 
tion in  this  excessively  difficult  matter. 

As  to  the  specific  treatment  of  already  fully  developed  cases  of  tabes,  in  such 
cases  in  which  a  combination  with  tertiary  nerve  syphilis  appeared  to  be  ex- 
cluded, I  formerly  did  not  use  mercury  and  only  in  the  last  six  years  have  I 
been  converted  to  the  use  of  mercurial  and,  of  late,  of  salvarsan  treatment  in 
certain  indications.  The  pains  and  crises  have  shown  themselves  most  certainly 
to  be  influenced  by  these  remedies ;  their  action  is  quite  frequently,  though  by 
no  means  always,  very  decided;  with  arsenobenzol  sometimes  a  striking  one.  In 
incipient,  or  at  least  in  pre-atactic  cases,  of  tabes,  with  regard  to  the  estimation 
of  the  influence  of  mercury  and  salvarsan,  great  scepticism  is  still  justified. 
Nevertheless,  I  have  the  impression  that  (in  cases  with  positive  Wassermann) 
the  course,  in  the  sense  of  mildness  (frequent  enough  otherwise,  however)  can 
be  influenced.  Evident  tendency  to  rapidly  progressive  ataxia,  as  well  as  poor 
condition  of  nutrition,  or  indeed,  "marantic  tabes,"  contraindicate  mercury,  not, 
however,  salvarsan,  while  on  the  other  hand,  this  last  may  be  dangerous  in  the 
combination  with  syphilogenic  diseases  of  the  heart  or  aorta.  An  unqualified 
contraindication  against  mercury  cures  is  presented  by  parasyphilitic  optic 
atrophy.  I  have  seen  several  cases  together  with  the  ophthalmologist,  Paul 
Knapp,  which  in  spite  of  the  advice  of  both  of  us,  were  subjected  to  energetic 
mercurial  treatment  and  rapidly  got  worse.  Knapp  could  indeed  show  four  times 
most  exactly  that  the  degenerative  process  in  the  optic  nerve  progressed  much 
more  rapidly,  and  that  upon  abandonment  of  this  treatment  the  progression  was 
again  slower. 

Into  the  particularities  of  anti-syphilitic  treatment  we  will  not  enter  here. 
The  most  important  points  neurologically  will  be  mentioned  in  the  description 
of  lues  cerebro-spinalis.  As  to  the  iodide  medication  so  much  used  in  tabes 
dorsalis,  it  comes  into  consideration  less  as  an  antiluetic  as  on  account  of  aiding 
the  circulation  through  its  reduction  of  the  viscosity  of  the  blood  which  has 
been  shown  and  proven  in  the  course  of  iodide  cures  {Midler,  Enada,  etc.),  per- 
haps, also,  thanks  to  a  certain  vasodilator  effect,  a  better  circulation  through, 
and  nutrition  of,  the  affected  regions  may  occur,  which  opposes  the  degenerative 
destruction.  Along  with  the  internal  administration  of  the  alkaline  iodides  (in 
doses  of  0.5  (gr.  7%)  3  or  4  times  a  day,  after  20  days,  at  least  a  10  day  pause 
before  repetition  of  the  cure)  I  toscd  with  advantage,  iodipin  injections  (twice 
a  week  10  <■(•  of  the  25  per  cent.  oil).  The  other  organic  iodine  preparations 
(sajodin,  iodostarin,  lipiodin,  iodglidine,  iodon,  iodtropin,  etc.)  give  less  favor- 
able results;  nevertheless,  they  can  sometimes  be  ordered  in  sensitive  patients  or 
for  those  demanding  a  change  of  medicine. 

When  we  look  over  the  drugs  recommended  in  tabes  dorsalis  I  might  mention 
'"lost  only  from  its  historical  interest,  silver  nitrate  (0.01   made  into  a  pill  with 


194  LECTURE    XII 

bolus  alba  3  times  a  day)  introduced  into  the  therapy  of  tabes  by  Wwnderlich  in; 
his  day,  although  it  is  often  enough  prescribed  in  a  routine  manner  still.  I  am 
convinced  that  it  does  not  have  the  slightest  action  and  at  most  can  come  into 
question  symptomatically  in  gastric  crisis  (as  a  drug  limiting  the  secretion), 
here,  however,  it  is  usually  not  retained,  but  promptly  vomited.  According  to 
the  original  instructions,  pills  of  silver  nitrate  should  be  given  for  months  at  a 
time.  There  is  in  this,  however,  the  danger  of  "argyria"  (blue-black  coloring- 
of  the  skin  and  mucous  membranes),  even  when  the  rule  not  to  exceed  a  total 
dosage  of  10  grm.  has  been  observed.  Also,  protargol  which  can  be  given  in  10 
times  the  dose,  can  well  be  dispensed  with,  as  can  oxide  of  zinc  (0.05  3  or  4  times 
a  day)  and  chloride  of  gold  and  sodium  (0.01  t.  i.  d.).  As  to  ergotin,  recom- 
mended by  Charcot,  it  is  certainly  no  specific  against  tabes,  but  sometimes  op- 
poses the  tabetic  bladder  paralj'sis  in  consequence  of  the  affinity  for  the  smooth 
muscles  of  the  pelvic  organs  also  responsible  for  its  action  upon  the  uterus. 

T$      Extract,  secalis  cornuti 2.5 

Pulvis  secalis  cornuti    5.0 

M.  fiat  Pil.  No.  L.  S.     lpillt.  i.  d. 

A  somewhat  efficient  general  effect  in  tabetics  is  exerted  by  strychnine,  how- 
ever; we  can  administer  it  in  pill  form  or  subcutaneously  (0.003  to  0.00(5  per 
day — gr.  /{>o  to  gr.  Yn\  per  day).  Tinctura  nucis  vomica'  is  also  a  favorite 
remedy : 

ty      Tinct.    nuc.    vom 10.0 

Tinct.  cincona;  co 20.0 

M.  S.    30  drops  t.  i.  d. 

An  excellent  combination  has  been  given  by  Erb  in  the  form  of  his  "tonic- 
pills." 

I£      Ferri  lactat, 

Extract,  cinchon.  aq aa     5.0   (gr.   Ixxv) 

Extract,  nucis  vom 0.8   (gr.  xii) 

Ext.  gentians,  qs.  ut. 
Fiat  pil.  No.  C. 

S.    2  pills  t.  i.  d.  p.  c. 

I  have  had  better  results  from  the  following: 

K^      Ferri  lactat .  4.0   (gr.   lx) 

Quinin  sulphat    3.0   (gr.   xlv) 

Extract,  nucis  vom 1.0   (gr.   xv) 

Extract,  valerian    T.O    (gr.    c) 

Fiat  pil.  No.  C. 

S.     2  pills  t.  i.  d. 


SYPHILOGENTC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     195 

Next  to  strychnine  the  arsenic  preparations  should  be  recommended:  some- 
times arsenic  cures  produce  a  manifest  improvement  of  the  nutrition  and  of  the 
general  condition,  which  occasionally  has  a  favorable  effect  upon  the  course  of 
the  disease.  In  cachectic  symptoms  intensive  subcutaneous  arsenic  medication 
is  especially  to  be  recommended.  In  order  to  avoid  repetition  I  would  remind 
you  of  what  was  said  under  the  therapy  of  multiple  sclerosis.  Specially  suited 
for  the  treatment  of  tabes  appears  to  be  the.  in  England  officinal,  iodide  of 
arsenic  (Arsenii  iodidum,  B.  P.)  in  the  form  of  Donovan's  solution. 

R-      Arsenii  iodid 0.1 

Hydrarg.  biniodid 0.2 

Potass,  iodid 2.0 

Aq.  dest 60.0 

.M.  S.     From  5  to  100  drops  gradually  increased,  in  water  2  or  3  times 
a  day. 

While  different  derivatives  of  phosphoric  acid,  for  example  lecithin,  calcium 
glycerophosphate,  nucleinic  acid  or  nucleinate  of  sodium,  phytin,  etc.,  can  be 
occasionally  tried,  I  can  express  myself  as  to  the  whole  opo-  and  organo-therapy 
of  tabes  unfavorably  only,  also  as  to  the  use  of  spermin,  cerebin,  etc.  From 
fibrolysin,  which  has  been  recommended  upon  theoretical  grounds,  in  tabes,  I  have 
equally  failed  to  see  any  result. 

The  attacks  of  pain  in  tabetics  frequently  call  urgently  for  symptomatic 
medicinal  treatment.  What  is  to  be  said  here  agrees  entirely  with  our  previous 
remark  as  to  the  drug  treatment  of  neuralgia,  hence  we  will  not  again  enumerate 
the.  different  anti-neuralgics  and  their  combinations,  but  will  refer  to  Lecture 
III  (page  57).  As  peculiar  to  the  tabetic  pains,  we  will  only  refer  to  their 
occasional  susceptibility  to  the  action  of  two  drugs  to  which  ordinary  neuralgias 
scarcely  ever  react,  namely,  methylene  blue  and  sodium  nitrite.  The  first  acts, 
perhaps,  through  its  histo-chemical  affinity  for  the  axis  cylinders ;  the  last  by 
relieving  vascular  spasm.  Methylene  blue  is  always  to  be  combined  with  pow- 
dered  nutmeg  in  order  to  prevent  irritation  of  the  bladder.  Sodium  nitrite  only 
acts  when  used  subcutaneous] y,  and  since  it  may  act  as  a  cardiac  poison,  is  to 
be  given  in   very  carefully  regulated  doses. 

I£       Methylene  blue 0.1     (gr.    \V.,) 

Powdered  nutmeg 0.5   (gr.  T1/^) 

M.    Divide  into  10  capsules.     S.    1   t.  i.  d. 

K      Sodii  nitritis 0.1-0.3 

Aq.  desi ' 10.0 

Should  be  sterilized.  S.  1.0  to  1.25  (15-20  minims)  daily  (carefully  in- 
creasing the  quantity  of  sodium  nitrite). 

The  too  ready  ordering  of  hypodermic  injections  of  morphine  is  to  be  un- 
qualifiedly condemned,  since  many  tabetics,  by  carelessness  of  the  physician  in 
tins  respect,  acquire  the  morphine  habit.     Further,  it  looks  as  if  the  use  of  mor- 


196  LECTURE    XII 

phine  in  tabetics  favors  the  occurrence  of  gastric  crises,  probably  because  mor- 
phine excites  the  secretion  of  gastric  juice.  Where  morphine  injections  must  be 
used  as  a  last  resort,  it  is  best  combined  with  atropin. 

For  the  gastric  crises  Kodari  has  recommended  the  combination  of  pantopon 
and  atropin.  ly  Pantopon,  0.2 ;  atropin  sulph.,  0.01 ;  aq.  laurocerasi,  10.0 ; 
10  to  15  drops  2  or  3  times  a  day.  Many  patients  with  gastric  crises,  however, 
do  not  tolerate  the  internal  administration  of  drugs  (for  example,  of  cerium 
oxalate  recommended  in  doses  of  grm.  0.1).  Here  suppositories  often  work  well, 
containing,  for  example,  codein  phosph.  and  extract,  belladonna,  aa.  0.05  (gr. 
^4).  These  last  are  to  be  used  also  in  intestinal  crises,  while  in  laryngeal  crises 
local  application  of  10  per  cent,  cocaine  solution  or  inhalations  of  amyl  nitrite 
are  to  be  tried.  (Pearls  of  nitrite  of  amyl  containing  each  5  drops  are  con- 
venient.) 

In  disturbances  of  potence  yohimbin  can  be  tried.  However,  if  in  the  dose  of 
5  to  10  drops  of  a  1  per  cent,  solution  three  times  a  day  (or  in  tablets  of  0.005 
each — gr.  Vvi),  the  effect  is  not  satisfactory,  it  must  be  given  up,  as  no  larger 
doses  should  be  used.  It  need  not  be  attempted  to  excite  the  libido  in  such 
cases  in  which  erection  is  no  longer  possible. 

We  will  now  leave  the  subject  of  the  medicinal  treatment  of  tabes  and  pro- 
ceed to  consider  the  exceedingly  important  physical  methods  of  treatment. 

For  the  use  of  electricity  there  is  quite  an  extended  field.  Of  galvanic  pro- 
cedures I  would  mention,  first,  the  stabile  application  to  the  spine  which  is  found 
beneficial  by  many  tabetics.  Two  large,  flat  electrodes  are  applied,  one  to  the 
neck  and  the  other  over  the  sacrum,  and  a  current  of  5  milleampercs  gradually 
introduced;  after  3  minutes  the  current  is  gradually  reduced,  the  poles  changed, 
and  the  procedure  repeated.  In  lancinating  pains  stabile  galvanization,  the 
anode  over  the  peripheral  nerve  trunks  as  described  in  Lecture  III,  can  be  tried. 
It  helps  only,  however,  in  a  very  small  number  of  cases.  For  this  indication, 
more  result  is  promised  by  energetic  faradization  with  the  wire  brush  acting  as 
a  derivative,  which  often  is  useful  for  the  paresthesias  also.  The  faradic  cur- 
rent is  further  applied  within  the  urethra  in  the  treatment  of  weakness  of  the 
bladder ;  a  flat  electrode  is  placed  over  the  lumbar  region  while  a  catheter  elec- 
trode is  passed  one  cm  into  the  urethra ;  the  individual  applications  should  last 
5  minutes,  the  current  being  regulated  so  that  it  is  felt  plainly  but  not  pain- 
fully. Finally,  the  favorable  effects  occasionally  obtained  from  the  high  fre- 
quency current  in  crises  and  lightning  pains  should  be  mentioned.  As  to  hydro- 
and  balneotherapy,  I  would  warn  you  most  decidedly  against  the  application  of 
the  various  cold  water  procedures.  The  rapid  deterioration  often  observed  in 
tabetics  who  have  sought  aid  in  "cold  water  institutions"  speaks  impressively 
against  these  measures.  Tabetics  are  also  to  be  warned  against  the  other  ex- 
treme, namely,  hot  baths,  douches,  etc.,  or  mineral  springs  of  high  temperature. 
These  remarks  need  to  be  modified  only  as  regards  a  number  of  local  methods 
of  applying  heat,  which  sometimes  act  favorably  against  some  of  the  paroxysmal 
symptoms,  for  example,  hot  packs  or  application  of  the  incandescent  light  to 
the  limbs  in  lightning  pains,  application  of  the  thermaphor  or  hot  poultices  to 
the  epigastrium,  or  to  the  lower  abdomen  in  gastric  and  intestinal  crises.  Of 
mineral   springs   the   carbonated   salt   waters   are   to  be  considered   principally 


SYPHILOGEXIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     197 

(Nauheim,  Oeynhausen,  Saratoga,  Now  York,  Ute  Springs,  Col.,  Paso  Robles, 
Cal.),  also  the  ordinary  salt  baths,  since  arrangements  for  the  artificial  addi- 
tion of  carl      ic  acid  are  available  almost  everywhere  (Rheinfelden  Ischl,  etc.). 

Further,  the  indifferent  thermal  waters,  as  those  of  Wildbad  in  Wurtemburg, 
Badenweiler,  Teplitz,  Virginia  Hot  Springs,  Byron  Hot  Springs,  Cal.  The 
temperature  of  the  baths  should  not  exceed  35°  C.  (95°  F.),  their  duration 
should  usually  be  limited  to  half  an  hour.  It  has  become  the  fashion  of  late  to 
bring  forward  the  radium  content  of  these  baths  in  order  to  explain  their 
favorable  action ;  this  is  quite  possible,  but  as  far  as  the  artificial  radium  baths 
(radiogen,  etc.)  are  concerned,  they  usually  fail  in  lancinating  pains,  and  in  the 
rare  cases  in  which  they  have  been  reputed  to  aid,  the  element  of  suggestion  can- 
not be  excluded.  Good  results  are  to  be  obtained  also  with  less  expensive  in- 
gredients for  the  baths,  for  example,  with  the  slightly  stimulating  pine  needle 
"ozofluin"  baths,  etc.  Prolonged  baths  at  35°  C.  without  any  addition  are 
alleviating  for  many  tabetics.  As  a  powerful  derivative  application  in  the  lan- 
cinating pains,  I  can  recommend  applications  of  warm  salt. 

Of  mechanical  procedures  the  so-called  "suspension  treatment"  formerly 
played  a  great  role  in  tabes  dorsalis.  Hanging  up  the  patient  by  means  of 
Glisson's  [or  Say  res* — Tr.]  suspension  apparatus,  affects  an  "extension  of  the 
spinal  cord,"  which  in  man}'  cases  exerts  a  favorable  effect  upon  the  crises  and 
attacks  of  pain.  It  has  shown  itself,  however,  very  dangerous,  in  that  softening 
of  the  spinal  cord  has  been  observed  after  the  suspension.  Also  the  modified 
proceeding  in  which  the  feet  of  the  patient  remain  on  the  floor  or  the  extension 
is  carried  out,  the  patient  lying  on  an  inclined  plane,  is  now  abandoned.  Where 
we  wish  to  exercise  a  mechanical  traction  upon  the  spinal  cord  or  the  posterior 
roots,  we  can  accomplish  this  by  having  the  patient  lie  upon  his  back  and,  draw 
his  knees  up  as  near  to  his  chin  as  possible,  then  keeping  them  in  this  position 
for  some  time  by  a  band  passing  around  the  neck  and  the  bend  of  the  knee.  In 
tain  'tic  irritative  symptoms,  drawing  off  a  few  cc  of  the  cerebro-spinal  fluid  by 
lumbar  puncture  sometimes  acts  favorably. 

The  most  important  aid  in  the  correction  of  tabetic  ataxia  is  the  compensa- 
torv  exercise  treatment  developed  by  Frenkel,  Leyden,  Goldscheider,  and  others, 
in  which  the  patient  under  the  control  of  his  eyes  attempts  gradually  to  learn 
again  to  accomplish  the  coordination  of  his  movements.  These  exercises,  how- 
ever, should  never  be  pushed  to  the  extent  of  fatigue  (which  Is  best  avoided  by 
watching  the  pulse,  since  in  many  tabetics  the  sense  of  fatigue  is  absent),  should 
be  given  up  when  the  ataxia  is  rapidly  increasing,  and  are  best  begun  whin  we 
have   received   the   impression   that   the   tabes   is   in  a   stationary   C lit  ion.      It   is 

entirely  unnecessary  to  use  the  complicated  apparatus  often  proposed;  most  of 
tin'  requisites  can  lie  improvised;  for  example,  the  atactic  is  placed  upon  a  com- 
fortable chair  and  is  requested  t*o  practice  touching  with  the  point  of  his  foot 
feme  numbers  which  have  been  written  on  the  floor  in  front  of  him  until  finally 
lie  can  do  tliis  on  command,  or  a  small  ladder  is  placed  before  him  (  Fig.  64),  the 

individual  rounds  of  which  he  [earns  to  touch  with  his  foot  (practice  of  eumetria 
in  vertical  movements).     Then  exercises  in  walking  are  taken  up.  in  which  the 

IV.t  of  the  patient   are  to  be  placed narked  areas;  for  this,  simple  lines  on  the 

floor  or  a  strip  of  linoleum  as   in   Fig.  <>."»  and   Fig.   <>(!  can  be  used.      A    further 


198 


LECTURE    XII 


stage  is  that  of  making  steps  of  different  length,  stepping  over  pieces  of  wood 
placed  at  regular  or  irregular  distances  apart,  of  like  or  unlike  dimensions,  etc. ; 
finally,  exercises  in  climbing  stairs.  For  the  upper  extremities  a  chessboard, 
whose  individual  squares  the  patient  must  touch  with  his  finger,  a  board  with 
holes  into  which  a  stopper  is  to  be  placed,  or  an  old  typewriter,  etc.,  can  be  used. 

In  treating  the  hypotonia  and  the  joint  troubles  in  tabes  we  may  need  the 
aid  of  the  orthopedist  to  apply  a  supporting  corset  in  relaxation  of  the  trunk 
muscles,  suitable  splints  in  genu  recurvatum  and  arthropathies.  Massage  should 
only  be  practiced  by  a  skilled  operator  under  the  direct  control  of  the  physician, 
otherwise  it  can  only  increase  the  hypotonic  and  arthropathic  deformities  and 
do  more  harm  than  good. 

As  a  surgical  procedure  for  the  relief  of  the  gastric  crises,  which,  however, 
on  account  of  its  great  danger,  should  only  be  considered  when  everything  else 


Fig.  65. 
Exercise  Treatment  in  Tabes  Dorsalis. 


has  failed  and  the  symptoms  are  excessively  intense  and  persistent,  posterior 
radicotomy  has  of  late  been  recommended  by  Mingazzini,  Forster,  and  others. 
This  is  the  intradural  or  extradural  resection  of  the  posterior  roots  D.  7  to  L.  1 
(in  several  stages).  Franke  has  proposed  as  a  less  dangerous  substitute  opera- 
tion, neurexaresis  of  the  corresponding  intercostal  nerves  in  which  the  posterior 
roots  are  evulsed  also,  Konig,  the  (in  any  case  little  dangerous)  production  of 
deep  anesthesia  by  injecting  the  nerves  in  question  with  0.5  per  cent,  novocain- 
suprarenin  solution  after  Brawn.  This  last  method  can  also  be  tried  for  the 
lightning  pains,  but  only  temporary  l-esults  can  be  expected.  For  perforating 
ulcer,  finally,  Jabovlay  has  proposed  an  operation  which  consists  in  dissecting 
out  the  femoral  artery  in  Scarpa's  triangle  on  the  affected  side  and  separating 
it  for  some  distance  from  its  vascular  sheath.  The  resulting  division  of  the 
vaso-constrictor  nerve  fibers  accompanying  this  vessel,  has  as  its  result  a  periph- 
eral vaso-dilation  which  has  repeatedly  led  to  the  healing  of  ulcers  which  had 
resisted  all  other  treatment. 

In  every  tabetic  the  most  minute  regulation  of  his  whole  manner  of  life  is 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     199 

of  extraordinary  importance.  The  patient  should  be  warned  against  the  damage 
which  every  physical  overexertion  may  do  him ;  walks,  should  be  broken  by 
periods  of  rest,  climbing  stairs  should  be  restricted  as  much  as  possible;  moun- 
tain climbing,  even  the  slightest,  should  be  forbidden ;  also  long  standing  is 
dangerous.  Excesses  in  every  direction,  particularly  in  regard  to  alcohol,  must 
be  prevented,  sexual  excitement  avoided.  Regular,  full,  but  unstimulating  diet, 
long  night's  rest,  frequent  passing  urine  at  regular  intervals,  combating  con- 
stipation (when  possible  by  diet);  upon  all  these  things  great  stress  must  be 
laid  if  we  wish  to  aid  in  fulfilling  the  conditions  contributing  to  a  mild  course  of 
the  disease.  Where  there  is  a  tendency  to  cachexia,  but  only  after  gastroin- 
testinal crises  have  passed  off,  full-feeding  cures  are  to  be  undertaken  (see  Lec- 
ture XXVIII).  Last,  but  not  least,  the  psychotherapy  necessary  for  every 
tabetic  should  be  considered.  Avoidance  of  the  tendency  to  depressive  fore- 
bodings and  to  exaggerated  anxious  self-observation  present  in  almost  all 
tabetics,  may  in  itself  relieve  organically  caused  difficulties,  since  psychogenic 
factors  often  reinforce  disease  symptoms  having  a  material  substratum  and 
very   considerably   increase  their   intensity. 


LECTURE    XIII 

The  Syphilogenic  Diseases  of  the  Central  Nervous 

System 

B.     The  Progressive  Paralysis  of  the  Insane.     (General  Paresis) 

With  this  important  disease  which  Bai/lc,  in  1822,  isolated  as  a  clinical 
entity  and  an  intimate  knowledge  of  which  has  been  furnished  us  by  the  works 
of  Falret,  Westphal,  Krafft-Ebing  and  others,  we  will  occupy  ourselves  in  this 
lecture  only  in  a  very  condensed  manner.  Namely,  we  cannot  attempt  to  give 
an  accurate  picture  of  the  fully  developed  disease  with  its  different  clinical  varie- 
ties, as  nearly  every  psychiatrist  has  an  opportunity  to  observe  it  in  institu- 
tions, hence  these  matters  can  only  be  considered  very  cursorily.  We  must,  how- 
ever, consider  quite  thoroughly  the  particularities  of  the  prodromal  and  early 
stages  of  progressive  paralysis,  the  measures  indicated  in  it,  and  the  points 
important  in  differential  diagnosis  coming  under  consideration,  since  all  this 
comes  especially  within  the  sphere  of  the  neurologist,  even  also  within  that  of 
the  practical  physician,  and  since  here  mistakes  in  diagnosis  and  other  errors 
(unfortunately  quite  frequent)  may  have  regrettable  consequences  for  the  pa- 
tient, his  family  and  the  community. 

The  etiology  of  "paresis"  (to  use  a  designation  sanctioned  by  custom)  has 
already  been  discussed  in  connection  with'that  of  tabes,  and  we  have  there  learned 
that  along  with  the  primordial  syphilitic  damage,  different  injurious  factors  in- 
herent to  civilization  are  to  be  considered  as  important  contributing  causes.  The 
steady  increase  in  morbidity  since  the  recognition  of  the  disease,  which  according 
to  nearly  all  psychiatrists,  has  occurred,  stands  in  close  connection  with  these 
facts,  though  as  far  as  I  know,  extended  statistics  proving  this  latter  statement 
beyond  a  doubt  have  not  so  far  been  furnished.  Also  we  should  not  forget  that 
to-day  progressive  paralysis  is  much  more  surely  and  on  this  account,  much 
more  frequently  recognized,  than  was  the  case  some  decades  ago.  Arnaud  has 
shown  that  paresis  occurs  about  4  times  more  frequently  in  cities  than  in  the 
country. 

Pathological  Anatomy 

Progressive  paralysis  has  nothing  to  do  with  "softening  of  the  brain"  as 
which  it  is  usually  designated  by  the  laity  (a  peculiar  persistence  of  the  erroneous 
term  which  the  psychiatrist  Parchappe  used  in  the  year  1838  "softening  of 
the  cortical  layer").  Rather  is  the  disease  character!"^!  macroscopically  by  a 
progressive  atrophy  of  the  cerebrum,  particularly  s  frontal  lobes ;  micro- 

200 


SYPHILOGEXIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     201 

scopically  by  the  gradual  loss  of  the  tangential  fiber  layer  and  of  the  ganglion 
cells  along  with  compensatory  glia  proliferation  in  the  cortex  cerebri  (particu- 
larly in  the  frontal  regions  and  in  the  island  of  lied)  ;  further,  by  alteration 
of  the  cortical  vessels  (ectasies  with  perivascular  or  adventitial  infiltration  with 
round  cells  and  with  the  specially  typical  plasma  cells).  In  many  cases  there 
is  added  degeneration  of  the  pyramids,  in  others,  the  systematic  degeneration 
of  the  posterior  roots  and  the  posterior  columns  of  the  spinal  cord  described  for 
tabes.  Only  where  the  last  mentioned  lesion  is  already  developed  in  the  early 
stages,  the  clinical  picture  of  "tabo-paralvsis"  occurs ;  in  the  terminal  stage 
on  the  other  hand,  more  or  less  marked  changes  in  the  posterior  columns  in  the 
upper  cervical  region  are  an  almost  regular  autopsy  finding,  but  clinically  ir- 
relevant. Without  exception  also  there  is  found  a  fibrous  lepto-meningitis,  the 
"arachnitis  chronica,"  which  Bayle  considered  the  basis  of  the  affection. 

PRODROMAL    STAGE 

The  beginning  of  the  disease  in  the  great  majority  of  cases  (over  80  per 
cent.)  falls  in  the  4th  and  5th  decades  of  life.  According  to  Obersteiners  obser- 
vations, in  56  per  cent,  the  disease  begins  between  the  36th  and  the  45th  year; 
the  "incubation  period"  which  has  elapsed  since  the  luetic  infection,  he  esti- 
mates upon  the  average  as  121/2  years,  the  shortest  period  in  these  cases  was  3, 
the  longest  32,  years.  Men  are  about  8  to  10  times  more  frequently  attacked 
than  women.  Infantile  or  juvenile  paresis  (that  is,  beginning  before  the  20th 
year)  is  excessively  rare  and  occurs  only  upon  a  basis  of  hereditary  syphilis. 

The  first  alterations  are  in  the  sphere  of  the  intellect  and  are  manifested  by 
defects  of  memory  (particularly  for  recent  events)  further,  by  reduction  of 
mental  capacity  and  by  marked  distraction  and  forgetfulness,  so  that  the  pa- 
tient, for  example,  loses  himself  on  the  streets  of  his  own  town,  or,  like  one  of 
my  parities,  who  repeatedly,  when  fishing,  walked  into  the  stream  without  his 
rubber  boots.  In  the  ethical  sphere  also,  there  is  often  alteration.  The  family 
complain  of  formerly  unknown  acts  of  neglect,  of  brutal  egotism,  of  continued 
irritability.  The  patient  is  careless  in  his  business,  slovenly  in  his  dress,  in- 
decorous, even  obscene,  in  his  conversation.  Physical  troubles  also  appear,  how- 
ever. Attacks  of  hemicrania  not  rarely  in  the  form  of  the  so-called  "ophthalmic 
migraine"  occur.  These  we  will  study  in  a  later  lecture  (Lecture  XXX).  If 
Inch  paroxysms  of  headache  occur  in  the  thirties  and  forties  in  people  who  have 
not  before  suffered  from  migraine  we  should  be  awake-  to  the  eventuality  of  a 
threatening  paresis.  The  same'  remark  applies  lei  vertigo,  feiling  of  pressure 
in  the  head,  sleeplessness  and  other  "neurasthenic"  symptoms  when  they  occur 
for  the  first  time  in  former  syphiHtics,  in  these  ominous  decades,  and  we  can 
find  no  causal  factors  for  acquired  neurasthenia  (see  Lecture  XXVIII).  Par- 
ticular] v  alarming,  however,  are  the  following,  it  is  true  not  very  frequent,  phe- 
nomena: Temporary  paresis  of  one  hand,  temporary  eye  muscle  paralyses  with 
diplopia;  temporary  pupillary  rigidity  or  anisocoria,  fleeting  nocturnal  enuresis. 
The  patient  usually  feels  ill  ami  has  a  sense  of  alteration  of  his  psychical  per- 
sonality. The  fear  of  threatened  "softening  of  the  brain"  sometimes  drives 
him  to  the  physician.     (        p  also  lead  to  suicide  or  a  suicidal  attempt.     How 


202  LECTURE    XIII 

valuable  in  confirming  the  diagnosis  in  this  stage  the  "four  reactions"  of  Nonne 
can  be  we  have  already  mentioned. 

THE    INITIAL    STAGE 

In  th>  initial  stage  there  occur  in  different  groupings  a  large  number  of 
objective,  mainly  somatic  anomalies  which  even  without  the  aid  of  biological 
methods  and  the  anamnesis  throw  a  clear  light  upon  the  nature  of  the  affection 
present.     These  are: 

1.  Pupillary  Alterations.- — The  extreme  importance  of  these  anomalies  in 
the  symptomatology  of  paresis  is  shown  from  the  fact  that  according  to  the 
statistics  of  Mignot,  Schrameck  and  Parisot  only  6  per  cent,  of  the  cases  present 
intact  pupils.  The  anomalies  of  iris  innervation  in  progressive  paralysis  are 
in  the  main  the  same  as  those  in  tabes  dorsalis,  and  after  the  very  careful  in- 
vestigation of  B  untie,  the  Argyll-Robert  son  phenomenon  (reflex  rigidity  or 
slowness)  occupies  by  far  the  first  position  in  frequency.  It  is  interesting,  too, 
that  Wolff,  Gaupp,  Reichardt  and  Bumke,  in  altogether  about  70  cases  of 
paresis  with  the  Argyll-Robert  son  symptom,  found  in  each  instance  changes  in 
the  posterior  columns  in  the  cervical  region,  that  is,  a  combination  with  tabes 
superior.  Fiirstner  and  Naka  have  indeed  occasionally  found  absolutely  intact 
posterior  columns  with  clinically  surely  recognized  reflex  pupillary  rigidity,  and 
we  are  hence  of  the  opinion  that  even  without  tabetic  complications  the  typical 
Argyll-Robertson  symptom  can  occur  in  paresis,  namely  in  the  initial  stage. 
The  cases  which  come  to  autopsy,  however,  have  almost  without  exception  reached 
the  terminal  stage,  and  we  have  already  remarked,  that  in  this  last,  the  pos- 
terior columns  of  the  upper  cervical  region  are  almost  never  intact.  Further 
pupillary  symptoms  of  paresis  are  absolute  immobility  (more  frequent  than  in 
tabetics),  irregularity,  abnormal  wideness  or  narrowness  (the  first  rarer  than 
the  latter),  anisocoria  (very  frequent),  and  the  so-called  "bounding  mydriasis," 
that  is,  a  rapid  variation  between  the  width  of  the  right  and  of  the  left  pupil. 
The  last  phenomenon  originally  considered  pathognomonic  for  paresis  and 
tabes,  occurs  also  in  Basedow's  disease  and  even  in  neurasthenia  and  hysteria. 

2.  Speech  Disturbances. — Already  in  1814  the  genius  Esquirol  wrote  in  a 
medical  lexicon  in  an  article  on  "Dementia,"  "Speech  difficulty  is  a  fatal  sign," 
and  it  is  not  to  be  doubted  that  he  had  in  mind  cases  which  since  then  have  been 
classed  with  dementia  paralytica.  Even  the  public  knows  that  "softening  of 
the  brain"  begins  with  trouble  with  the  tongue.  The  first  disturbances  usually 
present  themselves  in  the  form  of  the  so-called  "syllable  stumbling"  which  Kuss- 
muul  defined  as  follows:  "A  disturbance  of  the  coordination  of  the  whole  word  as 
a  speech  unit,  with  intact  formation  of  sounds  and  syllables."  The  patient  mis- 
places letters  and  syllables,  leaves  them  out,  or  repeats  them  in  the  course  of 
the  word  (perseveration),  confuses  them  with  similar  sounding  ones.  So,  for 
example,  for  artillery  brigade,  he  says  artrallery  brigade;  for  abnormality,  ab- 
normalty;  for  truly  rural,  tooly  looral,  etc.  If  he  is  required  to  repeat  these 
somewhat  complicated  words  several  times  it  is  noticed  that  each  time  the  dis- 
turbance, which  at  the  start  was  overcome  by  strained  attention,  becomes  more 
marked  and  finally  the  word  becomes  unrecognizable.     One  must,  however,  be 


SYPHILOGEMC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     203 

careful  with  this  test  since  the  more  educated  patients  may  so  be  bi-ought  to 
make  a  diagnosis  of  their  own  cases.  It  is  better  on  this  account  to  have  them 
read  a  newspaper  article  adapted  to  bring  out  the  defect  under  the  pretext  of 
testing  their  vision.  In  this  (as  well  as.  in  the  course  of  conversation  with  the 
patient)  the  following  ominous  speecli  disturbances  are  readily  noticed:  First, 
the  "drawling"  speech  in  which  it  is  noticeable  that  the  more  important  letters 
are  weakened,  p  is  pronounced  like  b;  t,  like  v  (the  form  of  the  mouth  is  natur- 
ally to  be  noticed  here),  particularly  that  r  is  pronounced  badly,  more  or  less 
like  1.  Further,  frequent  mistakes,  substitution  of  words,  paraphasias,  which 
the  patient  usually  does  not  observe  or  correct  (in  contradistinction  to  neuras- 
thenics or  mentally  fatigued  healthy  persons)  and  the  slow  and  hesitating  tempo, 
as  well  as  the  monotony  and  dullness  of  the  diction,  often  also  a  certain  nasal 
quality.  In  conversation,  finally,  the  patient  must  strikingly  often  stop  and 
think  of  a  word  or  phrase,  makes  many  grammatical  errors  and  mistakes  of 
syntax,  stalls  in  the  middle  of  a  sentence,  etc. 

3.  Motor  Irritative-  Symptoms. — In  the  first  place  the  exceedingly  char- 
acteristic fibrillary  twitching  in  the  region  of  the  lower  facial  should  be  men- 
tioned: this  either  plays  over  the  countenance  like  summer  lightning,  now  right, 
now  left,  now  about  the  comers  of  the  mouth,  now  over  the  chin,  or  spreads  as 
an  almost  continuous  fluttering  and  trembling  over  the  orbicularis  oris,  levator 
anguli  oris  and  quadratus  menti :  indeed,  occasionally  (on  emotion,  etc.),  it  ex- 
tends over  the  ala>  nasi  and  cheeks  up  to  the  eyelids.  Also  the  outstretched  tongue 
naves  and  trembles;  sometimes  there  are  recognized  in  it,  plainly,  gross  con- 
tractions of  the  larger  muscle  bundles.  In  the  muscles  of  the  extremities  anything 
analogous  is  scarcely  ever  perceived.  However,  tremor  of  the  fingers  is  very 
frequent.  It  is  not  very  characteristic  but  is  quick  and  fine,  increasing  upon 
excitement  and  exertion,  and  docs  not  cease  even  upon  complete  rest. 

•i.  Alterations  of  the  Handwriting. — Obersteiner  gives  the  following  in- 
b  Uigent  description  of  the  handwriting  of  the  paretic  in  the  initial  stage:  "The 
individual  type  of  the  characters  is  altered,  the  handwriting  is  often  smaller, 
sometimes  larger,  and  pointed;  it  appears  (analogue  of  the  hesitation  in  speech) 
not  as  from  a  copy,  often  markedly  artificial  with  unusual  flourishes,  etc.  Be- 
ginning affection  of  the  muscle  sense,  muscular  insufficiencies  manifest  them- 
■elves,  by  the  improper  use  of  light  and  heavy  strokes,  getting  off  the  line,  un- 
equal size  of  the  letters,  angular  excursions,  wavering  and  zigzag  strokes;  in 
addition,  leaving  out  of  words,  or  at  hast  of  syllables,  occasionally  doubling 
ol  the  latter,  mutilation  of  words,  to  paraphasic  phenomena." 

5.  The  "Paralytic  Fades."—  The  facial  expression  of  many  paretics  shows 
even  in  the  initial  stage  so  typical  a  picture  that  a  diagnosis  at  first  sight,  even 
among  the  crowd  in  the  reception-room,  is  not  infrequently  possible  for  the 
experienced  physician.  The  patient  looks  like  an  "all  nighter,"  sleepy,  relaxed, 
expressionless,  exhausted;  bis  face  has  about  it  something  dull  and  empty,  the 
action  of  his  muscles  of  expression  is  particularly  limited.  Asymmetric  inner- 
vation of  the  facia]  muscles  on  the  two  sides  (sometimes  varying,  so  thai  to  day 
She  right,  a  fev  days  later  the  left  naso-labial  fold  appears  more  marked),  is 
indeed  by  far  not  SO  frequenl  as  in  the  later  stages,  but  is  not  rarely  observed 
at  I  his   I  ime. 


204  LECTURE    XIII 


The  Further  Clinical  Picture 


Along  with  the  5  groups  of  symptoms  which  we  have  now  considered  and 
which  can  be  called  the  stigmata  proper  of  beginning  paresis,  the  clinical  picture 
otherwise  unfolds  itself  in  this  early  stage  in  the  following  manner: 

The  psychical  anomalies  which  impress  their  stamp  already  upon  the  pro- 
dromal stage  appear  now  in  much  more  definite  manner,  since  the  weakening  of 
judgment  and  beginning  disintegration  of  the  mental  personality  become  con- 
tinually more  evident. 

The  amnesia  for  recent  events  is  to  be  observed  in  much  higher  degree. 
Along  with  this,  there  is  from  time  to  time,  clouding  of  consciousness  which 
leads  to  occurrences  which  can  no  longer  be  considered  as  the  result  of  patho- 
logical forgetfulness  and  abstraction,  but  as  true  confusional  conditions.  In 
these,  things  may  be  done  which  bring  the  patient  into  conflict  with  the  police 
and  the  law,  for  example,  if  he  enters  a  strange  house  at  night,  begins  to  sing 
or  to  whistle  in  church  during  the  sermon,  urinates  on  the  pavement  of  a  crowded 
street,  etc.  On  the  other  hand  the  defect  in  ethical  inhibition  takes  the  upper 
hand  in  a  way  which  often  leads  to  forensic  consequences ;  particularly  often 
there  are  sexual  assaults  upon  children,  exhibitionism,  swindling,  defalcations, 
aggressive  behavior  in  public  places  and  on  the  street,  cheating  at  cards,  etc. 
A  teacher  treated  by  me  ran  amuck  among  his  pupils  with  a  pair  of  compasses. 
The  increased  feeling  of  self-importance  which,  in  the  fully  developed  disease  as- 
sumes the  classical  form  of  "grandiose  delusions,"  can  show  itself  even  in  the 
initial  stage  in  senseless  expenditure,  reckless  making  of  debts,  a  craze  for  in- 
vention, tendency  to  risky  undertakings,  etc.  Inability  to  criticise  his  own  acts 
or  those  of  his  surrounders,  testifies  to  the  growing  preponderance  of  intellect- 
ual defects.  The  physical  troubles  enumerated  for  the  prodromal  stage  either 
diminish  in  the  initial  stage  or,  on  the  contrary,  they  manifest  such  obstinacy 
and  intensity  (particularly  vertical  headache  and  sleeplessness)  that  they  are 
markedly  differentiated  from  the  same  symptoms  as  observed  in  neurasthenia. 
Occasionally  an  unconquerable  desire  to  sleep  by  day  is  in  contrast  with  the 
agrypnia  by  night.  Consciousness  of  illness  is  now  still  present  only  for  the 
physical  troubles,  not  for  the  mental  abnormality.  Not  rarely  there  appear 
even  in  the  initial  stage  those  ominous  paroxysms  from  which  only  a  minority 
of  paretics  are  spared,  but  which,  as  a  rule,  do  not  come  on  until  later  in  the 
disease,  and  which  are  known  under  the  name  of  "paretic  seizures."  We  dif- 
ferentiate apoplectiform  and  epileptiform  attacks.  In  the  first  the  patient  falls 
unconscious  as  in  a  true  attack  of  apoplexy,  and  shows  a  hemiplegia  after  he 
comes  out  of  the  coma.  This,  however,  entirely  disappears  within  a  few  days 
(occasionally  even  after  a  few  hours),  and  the  attack  is  accompanied  by  a  rise 
in  temperature  which  is  in  contrast  to  the  subnormal  temperature  of  cerebral 
hemorrhage  but  parallel  to  many  of  the  apoplectiform  paroxysms  of  multiple 
sclerosis.  Krafft-Elmig  has  pointed  out  a  further  differential  point  in  their 
diagnosis  from  cerebral  hemorrhage.  In  the  latter,  the  tendon  reflexes  in  the 
region  pai'alyzed  are  regularly  lost  for  about  2-4  hours  after  the  attack ;  in 
the  paretic  hemiplegias,  they  are  exaggerated  immediately  after  its  onset. 
Kraepelin  is  of  the  opinion  that  many  cases  of  death  at  middle  age  from  "apo- 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     205 

plexv"  are  really  examples  of  a  fatal  ending  of  apoplectiform  attacks  in  the 
initial  stage  of  paresis.  More  frequent  are  epileptiform  attacks,  -which  also  are 
often  accompanied  by  hyperthermia.  They  have  usually  the  character  of  the 
so-called  "Jacksonian  or  cortical  epilepsy"  which  we  will  study  in  a  later  lec- 
ture (XIX)  more  fully.  At  present  I  would  only  remark  that  they  consist  in 
tonic  and  clonic  spasms  which  usually  begin  in  one  corner  of  the  mouth  and 
spread  from  there  over  the  face  and  extremities  of  the  same  side  and  often  to 
the  other  side.  Sometimes,  however,  they  exactly  resemble  genuine  epilepsy: 
The  patient  falls  with  a  cry,  is  unconscious,  shows  general  tonic  and  clonic 
convulsions,  his  face  is  cyanotic,  fa?ces  and  urine  are  involuntarily  discharged, 
and  he  foams  at  the  mouth.  After  the  attack,  which  lasts  only  a  few  minutes, 
tin-  patient  is  confused  for  a  long  time  and  regains  entire  consciousness  only 
little  by  little.  In  connection  with  paretic  seizures,  both  apoplectiform  and 
epileptiform,  there  occasionally  occur,  usually  very  temporary,  aphasic,  or 
apractic  conditions,  symptom  complexes,  which  we  will  subject  to  a  thorough 
consideration  in  a  special  lecture  (XVIII). 

Changes  in  the  optic  nerve  corresponding  to  those  in  tabes  can  also  occur 
in  paresis,  but  this  is,  on  the  whole,  quite  rare.  Joffroy,  among  227  paretics, 
only  saw  more  or  less  developed  atrophy  of  the  papilla  27  times,  and  these  were 
usually  alterations  which  developed  only  in  the  later  stages. 

THE    STAGE    OF    THE    FULLY    DEVELOPED    PSYCHOSIS 

The  paroxysms  just  described  can,  in  that  they  leave  behind  a  considerable 
degree  of  mental  deterioration  which  further  rapidly  develops  into  dementia, 
mark  in  rather  precise  manner  the  passing  of  the  initial  stage  into  dementia 
paralytica  proper;  more  frequently,  however,  this  change  is  imperceptible. 
Where,  however,  the  disease  which  has  fully  developed  manifests  itself  in  de- 
lusional ideas  and  delirious  conditions,  the  beginning  of  a  new  and  more  severe 
stage  of  the  affection  is  recognizable  even  to  the  laity,  the  usual  consequence  of 
which  is  the  bringing  of  the  patient  under  psychiatric  care. 

In  the  usual  manner  this  paralytic  psychosis  will  now  be  subjected  to  a 
bather  schematic  classification  in  that  we  speak  of  a  depressive,  an  expansive, 
an  agitated,  and  a  dement  form.  If  it  is  always  remembered  that  between  these 
forms  there  are  intermediate  varieties,  and  that  they  even  can  pass  over  into  one 
another,  there  is  no  objection  to  using  didactically  these  divisions.  We  will 
hence,  adhering  closely  to  the  descriptions  of  Qberstevnm  and  Kraepelin,  present 
the  clinical  criteria  of  these'  4  chief  forms. 

1.  The  Depressive  (Melaneholie)  Farm. — Depressive  delusional  ideas 
which  show  lack  of  insight,  loss  of  power  to  criticise  and  which  cannot  be 
corn cted.  are  manifested,  often  iii  an  absurd  manner.  These  can  be  of  hypo- 
chondriacal nature  (the  patient.  Cor  example,  laments  that  his  head  is  shrunken, 
that  he  has  no  stomach,  that  his  body  has  turned  to  pus,  etc.),  or  they  can  take 
the  form  of  "micromania,"  having  committed  great  sins,  etc.  (The  patient 
complains  thai  he  has  stolen  a  million;  that  he  has  destroyed  his  family;  affirms 
that  he  is  dead  and  refuses  food,  since  a  corpse  cannot  eat.)  Into  the  course 
of  this  depressive   form,  conditions  of  maniacal  exaltation   with  grand  delusions 


206  LECTURE    XIII 

may  force  their  way,  and  when  the  alternation  is  regular,  we  speak  of  the  "cir- 
cular form"  of  paresis. 

2.  The  Expansive  ("Classic'")  Form. — In  this  variety,  to  which  the  first 
descriptions  of  the  disease  applied,  grandiose  ideas,  increasing  to  the  im- 
measurable, dominate  the  picture.  While  the  patient  first  contents  himself 
with  declaring  that  he  is  the  richest,  the  handsomest  and  the  healthiest  man  in 
the  country  ;  before  long  he  takes  himself  for  a  king,  an  emperor,  God,  Supergod ; 
he  is  going  to  marry  princesses ;  to  buy  Australia ;  he  is  building  a  railroad  to 
the  moon,  etc.  One  of  my  patients,  immediately  after  the  epileptiform  attack 
which  ushered  in  his  psychosis,  wrote  to  the  King  of  the  Greeks  to  urge  him  "in 
the  name  of  the  Swiss  people  gathered  on  the  .shores  of  the  Lake  of  Zurich"  to 
make  a  republic  out  of  Crete. 

3.  The  Agitated  (Maniacal)  Form. — This  variety  is  characterized  by 
conditions  of  maniacal  and  delirious  excitement.  The  patient  disturbs  day  and 
night,  roars  and  sings,  holds  confused  conversations  marked  by  flight  of  ideas, 
sleeps  hardly  any,  loses  flesh  rapidly;  attacks  of  real  frenzy  and  dangerous 
assaults  upon  his  surrounders  also  occur.  The  most  severe  cases  of  agitated 
paresis  have  been  called  "Galloping  Paralysis."  In  these  the  patient  after  a 
few  weeks  of  the  most  extreme  excitement,  obstinate  refusal  of  nourishment, 
complete  filthiness  and  absolute  sleeplessness,  perishes  from  heart  weakness  or 
from  dysenteric  diarrhoea. 

4.  The  Dement  Form. — Under  this  name,  we  include  all  the  cases  in  which 
from  the  start  progressive  dementia  stands  in  the  foreground  of  the  psychical 
symptomatology.  Amnesia  stretches  continually  farther  backward  until  finally 
his  whole  previous  existence  with  all  its  impressions  and  experiences  are  oblit- 
erated from  the  memory  of  the  patient.  Orientation  for  time  and  for  place 
suffers  and  is  finally  lost  entirely.  The  power  of  speech  is  impaired  more  and 
more,  not  only  on  account  of  the  increase  of  the  syllable  stumbling  and  the 
anomalies  occurring  even  in  the  initial  stage,  but  .also  through  the  progressive 
extinction  of  the  speech  memories.  "Amnesic  aphasia"  of  the  most  extreme  de- 
gree so  occurs.  Since  also  the  "conceptions  of  movement"  are  defective  an 
"ideational  apraxia"  goes  hand  in  hand  with  this  last.  The  gait  also  is  slow, 
clumsy,  uncertain ;  there  is  incontinence  of  urine  and  fa»ces. 

Remissions  and  Intermissions 

The  severe  disease  pictures  just  described  can  (sometimes  even  suddenly, 
almost  from  one  day  to  another)  so  far  disappear  that  the  patient  and  his 
family  get  the  impression  of  a  cure,  while  we,  in  such  cases,  usually  speak  only 
of  remissions,  occasionally,  however,  of  true  intermissions.  In  the  remissions 
a  return  to  the  psychical  and  somatic  status  of  the  initial  stage  usually  occurs. 
Sometimes,  however,  there  remain  more  or  less  marked  speech  disturbances, 
and  regularly  indeed  the  pupillary  anomalies  formerly  present.  During  these 
remissions  which  last  for  weeks  and  months,  seldom  for  years,  the  patients  are 
often  let  out  of  institutions  by  psychiatrists  and  many  times  take  up  their  call- 
ings again.  It  is  of  capital  importance  that  they  remain  under  permanent 
and  careful  medical  observation ;  only  too  frequently,  however,  they  are  with- 


SYPHILOGEMC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     207 

drawn  by  their  friends  from  such  oversight  until  a  paretic  seizure  or  the 
sudden  recurrence  of  their  delusional  ideas  again  bring  to  the  front  the  great 
seriousness  of  the  situation  and  dissipate  the  beautiful  illusions  as  to  cure.  I 
have  so  far  only  seen  paretics  of  the  expansive  and  depressive  type,  who  have 
been  let  out  of  the  asylum  on  account  of  remissions,  return  to  my  care;  the 
longest  of  these  remissions  lasted  one  and  one-half  years.  Frequently  I  have 
been  able  from  return  of  the  agrypnia,  slight  speech  disturbances,  pathological 
irritability,  headache,  unreasoning  desire  to  speculate,  etc.,  to  diagnose  the 
threatened  return  of  progression  of  the  disease  and  to  put  the  patient  back  in 
the  asylum  in  time;  in  one  case  in  which  the  wife  of  the  patient  could  not  be 
convinced  of  the  necessity  of  this  measure,  there  broke  out  on  the  next  day  a 
condition  of  extreme  excitement  with  tendencies  to  violence  and  to  suicide,  in 
which  dreadful  results  were  only  with  great  difficulty  prevented.  One  should 
also  exercise  continued  control  over  paretics  during  these  remissions  since  it 
is  not  only  one  of  the  most  thankless  tasks,  but  also  one  of  the  most  responsible 
positions  in  which  the  physician  can  be  placed.  The  great  frequency  of  remis- 
sions in  the  classical  form  of  progressive  paralysis  is  emphasized  by  most  psy- 
chiatrists; in  the  agitated  form  they  are  rare,  in  the  dement  form  they  almost 
never  occur. 

True  intermissions  of  j-ear-long  duration  are  not  entirely  rare.  My  teacher. 
L.  WiUe,  told  me  about  one  of  his  paralytic  patients,  who,  during  an  inter- 
mission, was  able  to  assume  again  his  position  as  minister  of  a  small  German 
state.  A  patient  of  Kracpel'uis  not  only  discharged  his  duties  as  employee  in 
the  telegraph  office  for  5  years,  but  rose  to  higher  positions,  stood  examinations 
and  married.  Another  one  who  presented  grandiose  delusions,  speech  disturb- 
ance, pupillary  rigidity,  WesiphaVs  symptom,  and  attacks  of  vertigo,  lost  his 
delusional  ideas  and  could  again  occupy  his  former  position  as  janitor  at  a 
school  for  0  years.  A  patient  of  Tuczck,  who  in  his  grandiose  delusions  de- 
clared himself  Pope,  Emperor  of  Germany,  King  of  Darmstadt  and  father  of 
187  boys  born  at  the  same  time,  who  presented  the  characteristic  somatic  stig- 
mata of  paresis,  and  after  repeated  paretic  seizures,  had  already  passed  into  a 
condition  of  advanced  cachexia,  little  by  little  recovered  completely  both  physi- 
cally and  mentally,  took  up  again  his  position  as  conductor,  and  5  years  after 
the  beginning  of  his  intermission  was  promoted  "on  account  of  exceptionally 
good  work,"  then  2  years  later  had  to  give  up  his  position  finally  because  of 
'•talus."  Only  just  before  his  death,  20  years  after  the  beginning  of  the  inter- 
mission, he  presented  again  mental  anomalies,  which  however,  Knoblauch,  under 
whose  care  he  had  come,  considered  only  as  "marantic  confusion."  The  autopsy 
showed  in  the  brain  none  of  the  alterations  characteristic  for  progressive  paraly- 
sis. It  was  otherwise  in  a  case  of  Dobrschansky's,  in  which  after  an  intermis- 
sion of  15  years,  practically  amounting  to  recovery,  the  autopsy  showed  typical 
paralytic  alterations. 

THE    TERMINAL    STAGE 

However  different  the  clinical  pictures  of  florid  paresis  may  be,  the  patients 
finally  in  the  end  stage  all  come  into  the  same  wretched  condition.  The  de 
lusions  have  disappeared   in  the  predominating  dementia,  the  patient   has  lost 


208  LECTURE    XIII 

all  mental  relations  with  the  external  world,  even,  indeed,  the  consciousness  of 
his  own  personality,  his  movements  are  now  only  rudimentary,  his  discharges 
are  all  passed  in  the  bed,  he  utters  only  inarticulate  sounds,  must  be  fed,  the 
heart's  action  grows  feeble,  the  skin  is  anesthetic,  livid,  cedematous,  trophic  dis- 
turbances, bed-sores,  abscesses,  hypostatic  and  aspiration  pneumonias  develop, 
and  put  an  end  to  a  vegetative  existence  hardly  any  longer  to  be  considered  as 
"life,"  if  death  does  not  occur  from  paralysis  of  the  heart  in  a  paretic  seizure. 
In  the  end  stage,  "status  epilepticus"  from  an  unbroken  series  of  attacks  is 
frequent. 

Prognostic 

Cases  like  those  of  TuczeTc  and  Knoblauch,  are  such  rare  exceptions  that 
they  cannot  prevent  us  from  making  the  general  statement,  that  general  paresis 
leads  inexorably  to  death ;  besides,  the  autopsy  findings  in  these  patients  also 
do  not  exclude  the  fact  that  there  are  particularly  complete  and  long  intermis- 
sions, during  which  the  patient  may  be  taken  away.  It  is  certain  further,  that 
in  many  of  the  so-called  "recovered  pareses"  the  condition  was  really  some  other 
symptomatologically  closely  related  affection  (alcoholic  pseudo-paralysis,  cere- 
bral syphilis  with  psychosis,  etc.).  The  proximate  causes  of  death  can  be:  in 
the  early  stages,  and  further,  at  the  height  of  the  agitated  and  depressive  forms, 
suicide;  in  all  stages,  paretic  seizures;  in  the  terminal  stage,  aspiration  or 
hypostatic  pneumonias,  ascending  infection  of  the  urinary  tract,  sepsis  from 
bed-sores  or  injuries,  asphyxia  from  "choking,"  finally,  simple  marantic  heart 
failure. 

Between  the  individual  clinical  varieties  there  is  a  certain  difference  in 
malignancy.  Relatively  more  favorable  is  the  "Classical,"  the  expansive  form; 
in  it  remissions  may  most  frequently  be  hoped  for,  the  paralytic  attacks  which 
almost  always  hasten  the  progress  of  the  disease  are  comparatively  rare,  and 
finally,  long  stationary  periods  occur.  So,  then,  this  form  can  here  and  there 
present  quite  a  long  duration  of  the  disease  (to  about  15  years)  ;  in  general, 
however,  from  the  beginning  of  the  grandiose  ideas  to  death,  a  period  of  about 
3  years  can  be  predicted.  The  other  forms  usually  lead  in  less  than  2  years 
to  death ;  apart  from  the  "galloping"  variety  of  the  agitated  form,  the  dement 
type  is  most  malignant,  since  in  it,  the  paretic  seizures  are  very  frequent  during 
the  whole  time  of  the  disease  and  remissions  are  almost  entirely  excluded. 

Differential  Diagnosis 

In  the  prodromal  stage  the  differentiation  of  progressive  paralysis  from 
neurasthenia  can  be  a  matter  of  great  difficulty  when  it  concerns  a  patient  who 
has  had  syphilis.  Not  a  few  former  syphilitics  become  neurasthenic  on  account 
of  fear  of  paresis.  It  has  been  asserted,  however,  often  with  too  great  positive- 
ness,  that  this  nosophobia  speaks  against  paresis,  since  in  this,  prodromal  auto- 
diagnosis  are  not  at  all  rare.  It  is  notable  on  the  other  hand,  that  the  neuras- 
thenic occupies  himself  much  more  with  his  troubles  and  observes  them  much 
more  carefully  and  in  detail,  notes  them  down  and  describes  them  to  the  physi- 
cian more  frequently,  than  the,  even  at  the  start,  rather  indolent  paretic,  who  is 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     209 

generally  indifferent  and  forgetful  as  to  the  changes  in  his  own  personality. 
Plain  alterations  of  character  for  the  worse,  with  transgressions  against  pro- 
priety and  custom,  speaks  strongly  for  paresis.  Further,  the  "weakness  of 
memory"  complained  of  by  neurasthenics  on  careful  testing,  shows  itself  to  be 
only  the  expression  of  the  imperfect  and  abnormally  fatigable,  power  of  mental 
concentration,  while  that  of  the  paretic  represents  a  true  dys-  or  amnesia  for 
recent  events  which,  indeed,  is  plainer  objectively  than  subjectively.  Loss  of 
topographic  memory  pictures,  getting  lost,  never  occurs  in  neurasthenia.  Very 
properly,  on  the  other  hand,  Oppenhcim  suggests  that  imperative  conceptions 
speak  decidedly  against  paresis.  Very  suggestive  of  the  latter  are  temporary 
paresis  or  nocturnal  enuresis.  With  entrance  into  the  initial  stage  with  its  char- 
acteristic somatic  anomalies  and  the  increasingly  more  evident  psychical  dis- 
turbances, the  differential  diagnosis  soon  loses  its  difficulty,  particularly  when 
a  paretic  seizure  roughly  tears  down  the  last  veil.  We  must  not  forget  here 
to  allude  again  to  the  great  value  of  the  "4  reactions"  of  Nonne. 

Also  when  the  diagnosis  between  hysteria  and  beginning  paresis  is  difficult 
the  chemical,  cvtological  and  serological  blood  and  spinal  fluid  examinations 
can  greatly  aid  us.  As  an  example  I  would  mention  to  you  a  case  described  by 
Jusgen  which  also  is  important  medico-legally  since  not  rarely  a  head  injury 
gives  the  signal  for  the  outbreak  of  progressive  paralysis.  It  was  that  of  a 
workman  who  was  struck  upon  the  head  by  a  piece  of  falling  timber  while  in 
apparently  good  health  and  in  whom,  after  about  4  weeks,  mental  alterations 
not  rare  in  traumatic  neuroses  appeared ;  now  indifference,  now  labile  "affects" 
and  nervous  complaints.  Clinical  examination  showed  "hysterical"  convulsions 
which  partially  could  be  relieved  by  suggestion;  on  the  other  hand  no  positive 
signs  of  progressive  paralysis.  Nevertheless,  blood  examination  showed  a 
strongly  positive  W assermarm  reaction,  as  did  also  that  of  the  spinal  fluid 
with  increased  globulin  and  pleocytosis.  The  further  course  of  the  disease  justi- 
fied these  reactions;  during  the  first  weeks  of  observation,  indeed,  the  hysterical 
symptoms  remained  fully  developed,  namely,  there  was  great  suggestibility. 
Then,  however,  there  was  rapid,  unmistakable  change;  negativism  was  sub- 
stituted for  suggestibility,  the  patient  became  restless,  spoke  in  monotone,  mani- 
fested foolish,  depressive  ideas  and  passed  into  a  marked  paralytic  stupor. 

The  differential  diagnosis  from  multiple  sclerosis  was  already  discussed  in 
connection  witli  this  last  (page  139).  That  from  the  so-called  "pseudo-paralv- 
.si>"  (of  luetic  and  arteriosclerotic  nature)  as  well  as  from  certain  manifesta- 
tions of  brain  tumor,  will  be  taken  up  in  later  lectures  (XIV,  XV,  XIX).  We 
must  not  neglect  to  consider  at  this  point  "sleeping  sickness,"  all  the  more, 
since  this  affection,  as  Spielmeyer  has  recognized,  not  only  has  clinical  analogies 
with  progressive  paralysis,  but  also  etiological  relations  (similarity  between 
fcrypanosome  and  spirocha'te  infection)  and  a  certain  pathologico-anatomical 
agreement.  Along  with  the  somnolence,  this  endemic  disease  of  the  black  por- 
tion of  the  earth's  population,  which  is  called  after  its  most  striking  symptom, 
shows  frequently  disturbances  of  motility  and  of  the  reflexes,  epileptiform  at- 
tacks, speech  disturbances,  decrease  in  intelligence,  weakness  of  memory,  de- 
lusional ideas;  besides  this  lymphocytosis  of  the  spina]  fluid.  In  Africa  the 
differential  diagnosis  between  these  two  conditions  can  sometimes  be  difficult, 


210  LECTURE    XIII 

namely  when  the  "sleeping  sickness"  (which  indeed  is  seldom  the  case)  attacks 
a  white  person. 

Treatment 

On  account  of  our  inability  to  permanently  arrest  the  disease  process,  as  soon 
as  the  diagnosis  is  confirmed,  we  must  endeavor  to  delay  the  development  of  pro- 
gressive paralysis  as  much  as  possible  and  to  favor  the  occurrence  of  remissions. 
In  the  prodromal  and  initial  stages  the  most  important  point  is  immediate  re- 
moval from  business  activity  and  transference  to  as  quiet  and  nonirritating 
surroundings  as  possible,  for  example,  such  as  are  found  in  well  managed  sani- 
taria. You  cannot  be  too  urgently  warned  against  sending  such  patients  to 
hydrotherapeutie  institutions  (there  are  unfortunately  too  many)  where  the 
patients  are  subjected  to  fixed  cold  water  procedures  and  incipient  paretics  are 
thereby  greatly  injured.  If  in  a  neurasthenic  condition  there  is  even  the  slight- 
est suspicion  of  paresis  in  the  prodromal  stage,  care  should  be  taken  that  the 
patient  is  not  given  cold  baths  or  douches.  Great  importance  is  to  be  attached 
to  full  but  simple  and  nonconstipating  diet,  with  avoidance  of  all  stimulants 
(alcoholics,  spices,  tea,  coffee).  For  sleeplessness  drugs  must  often  be  resorted 
to  (veronal,  0.5—1.0  (gr.  8—15);  trional,  1.0—2.0  (gr.  15—30;  scopolamin, 
0.0005  (gr.  Vioo);  paraldehyd,  3.0-5.0  (oi-oii)  (greatly  diluted);  amylen- 
hydrate,  2.0—4.0  (."  ss.— 5i),  (greatly  diluted),  may  be  alternated  as  necessary). 
No  time  should  be  wasted  on  baths,  sponging,  or  psychotherapy;  if  the 
tendency  to  agrypnia  is  reduced,  the  hypnotics  may  be  gradually  limited  or 
withdrawn,  although  in  this  case  they  represent  the  less  of  two  evils,  since  we 
must  unceasingly  attempt  to  procure  rest  for  the  diseased  brain  during  the 
night. 

The  question  as  to  whether  antisyphilitic  treatment  should  be  instituted  or 
not,  cannot  be  answered  in  general  terms.  Many  psychiatrists  and  neurolo- 
gists, as  Krafft-Ebing,  Kraepclin  and  Dejerlne,  consider  mercurial  treatment  as 
contraindicated  when  the  diagnosis  is  certain,  since  it  reduces  the  nutrition  of 
the  patients  and  in  a  metasyphilitic  disease  can  accomplish  nothing.  Others, 
as  Leredde  and  Ziehen,  on  the  contrary,  are  of  the  opinion  that  under  mer- 
curial treatment  remissions  oftcner  occur,  are  more  marked  and  last  longer  than 
without  such  treatment.  Ziehen  hence  advises  the  trial  in  every  incipient  case 
of  a  course  of  inunctions  with  subsequent  administration  of  iodides,  and  even 
in  advanced  cases  he  has  an  inunction  made  twice  a  week  and  gives  0.2  (gr.  3) 
iodide  of  sodium  a  day.  I  consider  an  intermediate  standpoint  correct,  and 
limit  the  mercurial  treatment  to  early  cases  which  have  not  previously  been 
sufficiently  mercurialized,  that  is,  when  they  give  a  decidedly  positive  Wasser- 
mann  reaction ;  further,  I  avoid  too  energetic  treatment,  and  content  myself 
with  a  series  of  10  to  12  intramuscular  injections  of  about  1  cc  of  the  following 
solution:  Hydrarg.  biniodid,  0.2;  sodii  iodid,  0.2;  sodii  chlorid.,  0.075;  aq. 
dest.  10  cc.  (The  series  can  be  repeated  later.)  I  have  certainly  never  clone 
any  harm  with  these  injections  (given  at  intervals  of  from  1  to  2  days),  but 
believe  that  I  have  occasionally  aided  in  the  establishment  of  remissions.  As 
to  salvarsan,  with  which,  however,  I  have  had  but  very  limited  experience,  I 
can  neither  affirm  the  one  thing  nor  the  other.     Since  I  have  in  two  instances 


SYPHILOGEXIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     211 

gained  the  impression  that  it  accelerated  the  disease  process  or  provoked  seiz- 
ures and  have  had  similar  experiences  narrated  to  me  by  colleagues,  I  advise 
on  principle  against  this  otherwise  so  valuable  remedy,  in  paresis.  Only  in  one 
case  was  there  improvement,  and  this  was  only  in  the  subjective  symptoms, 
while  speech  disturbances,  facial  tremor,  sluggishness  of  the  pupils,  intellectual 
weakness,  etc.,  were  not  influenced  in  their  progress  by  the  injections. 

Into  the  symptomatic  therapy  and  care  of  the  fully  developed  disease  and 
of  its  terminal  stage  (sedative  drugs,  keeping  clean  those  who  suffer  from  in- 
continence, the  prevention  and  treatment  of  bed-sores,  tube  feeding  in  refusal 
of  food,  when  there  is  danger  of  choking,  etc.)  we  need  not  enter;  these  things 
are  mainly  for  the  asylum  psychiatrist.  It  may  be  said,  however,  that  for 
getting  a  disturbed  paretic  into  the  asylum,  a  large  dose  of  scopolamin  hydro- 
brom.  (0.001  =gr.  1/60)  injected  subcutaneously,  exerts  a  strikingly  sedative 
effect  (''chemical  camisole"). 

In  conclusion,  a  few  words  upon  some  new  and  interesting,  although  yet 
incomplete,  therapeutic  experiments.  These  are  based  upon  the  familiar  ex- 
perience that  remissions  in  progressive  paralysis  occur,  not  very  infrequently, 
after  febrile,  infectious  and  suppurative  processes.  Upon  this  was  based  the 
old  methods  of  provoking  suppuration  by  the  inunction  of  Autenrieth's  "Pus- 
tulating ointment"  (ung.  tartari-stihiati)  or  by  vaccination.  Of  late  the 
Vienna  psychiatrist  Wagner  v.  Jauregg  has  advised  the  systematic  use  of  tuber- 
culin injections,  upon  the  favorable  effects  of  which  with  regard  to  the  oc- 
currence of  remissions  and  in  prolongation  of  life  in  paretics,  Piles  and  Dobr- 
schansky  have  reported.  Beginning  with  a  dose  of  from  0.01  to  0.1  of  tuber- 
culin, the  dose  is  gradually  raised  to  0.5;  this  last  dose  is  given  7  to  12  times, 
each  injection  at  the  interval  of  about  2  days.  The  raising  of  the  dose  is 
dependent  upon  the  degree  of  febrile  reaction;  temperatures  of  over  39° 
(103.1°  F. )  are  avoided  as  far  as  possible.  Sometimes  Wagner  precedes  the 
tuberculin  cure  by  antiluetic  treatment.  Friedlander  has  reported  favorable 
results  from  dead  cultures  of  colon  and  typhoid  bacilli.  O.  Fischer  and  Donath 
produce  in  paretics  an  artificial  hyperleucocytosis  by  injections  of  nucleinic 
acid;  the  latter  uses  the  following  solution:  1}  sodii  nucleinat.,  sodii  chlorid., 
Is  '_'.();  aq.  dest.  steril.,  100.  M.  S.  use  for  one  or  two  injections.  The  in- 
jections, carried  out  at  Intervals  of  from  5  to  7  days,  should  produce  a  leu- 
coevtosis  of  from  10,000  to  25,000  lasting  some  time.  Sometimes  they  produce 
decided  local  reaction.  Donath  found  in  70  per  cent,  of  the  cases  treated  more 
nr  [ess  marked  improvement. 

The  recent  discovery  by  Noguchi  .-mil  Mnin-r  of  spirocha'br  in  tlir  brain  substance  in  gen- 
eral paresis  (confirmed  by  other  observers)  semis  to  settle  the  question  of  the  relation  between 
this  disease  and  syphilis.  Very  recently  Noiiiirhl  has  reporteil  finding  spirochaetae  in  the  spinal 
cord  in  one  ease  of  tabes  out  of  twelve  examined.  In  view  of  these  discoveries,  it  has  been  urged 
by  sonic  writers  that  the  terms  "inrta-"  and  "para-syphilitic"  be  abandoned.  In  so  far,  how- 
ever, as  they  are  used  to  imply  certain  pathological  changes  differing  from  those  usually  consid- 
ered characteristic  of  syphilis,  they  may  well  be  retained. 

On  account  of  the  inaccessibility  of  the  spirochetal  in  the  central  nervous  system  to  sal- 
rersan  introduced  into  the  circulation,  Bvrift  and  Elli»  have  recommended  administering  this 
remedy  intravenously,  one  hour  later,  bleeding,  cent ri filiating  the  blood,  beating  the  scrum  for 
one  hour  to  5fi°  ('.,  letting  stand  on  Ice  overnight,  making  a   tn  per  cent,  dilution  of  this  serum 


212  LECTURE    XIII 

with  physiological  salt  solution,  and  after  preliminary  lumbar  puncture  and  withdrawal  of  a 
corresponding  amount  of  fluid,  injecting  into  the  spinal  canal  15  to  330  cc  of  this  "salvarsanized 
serum."  It  is  thought  that  by  this  means  the  salvarsan  in  the  blood  may  be  brought  directly 
into  the  ventricles  and  lymph  spaces,  and  can  attack  the  spirochaetae  there  harbored.  While 
this  method  has  been  used  to  a  considerable  extent  in  the  United  States,  it  has  not  entirely 
fulfilled  the  hopes  attached  to  it,  and  our  experience  with  it  is  as  yet  insufficient  to  warrant  a 
positive  opinion  as  to  its  value.  The  direct  injection  of  salvarsan  into  the  spinal  canal  has 
been  tried,  but  some  serious  results  have  followed  this  method  of  administration,  and  its  tech- 
nique is  as  yet  insufficiently  developed,  hence  it  can  hardly  be  recommended  at  the  present  time. 
— Translator. 


LECTURE  XIV 

The  Syphilogenic  Diseases  of  the  Central  Nervous 

System 

Gentlemen  :  Now  that  in  the  last  three  lectures  we  have  studied  the  two 
most  typical  representatives  of  the  metasyphilitic  nervous  diseases,  we  will  take 
up  to-day  the  consideration  of  syphilogenic  affections,  which  in  pathologico- 
anatomical  and  symptomatological  relation  present  less  uniformity.  These 
are,  1,  the  so-called  syphilogenic  combined  system  diseases  and  2,  cerebro- 
spinal syphilis. 

C.     Syphilogenic  Combined  System  Diseases 

In  Lecture  VIII  we  have  seen  in  Friedreich's  disease  a  representative  of  the 
so-called  combined  system  diseases,  but  put  off  the  general  consideration  of 
these  in  no  way  frequent  disease  forms  until  later.  It  is  now  time  to  enter  into 
a  short  discussion  of  this  nosological  conception. 

While  in  spinal  hereditary  ataxia  there  is  the  combination  of  a  purely 
endogenically  caused  degeneration  in  the  different  sets  of  fiber  systems  of  the 
posterior  and  lateral  columns  of  the  spinal  cord,  similar  lesions  can  occur  from 
other  causes.  First  from  the  action  of  poisons  or  toxines  which  are  elective, 
but  which  affect  at  the  same  time  several  categories  of  long  tracts  and  which 
lead  to  the  progressive  degeneration  of  the  neurones  affected.  Next,  as  par- 
ticularly Nonne  and  Friind  have  shown,  disseminated  focal  diseases,  starting 
from  the  vascular  apparatus,  which  is  situated  within  the  areas  of  definite  fiber 
systems  (for  example,  the  pyramidal  tracts,  or  the  columns  of  Goll)  which  in 
consequence  of  the  descending  or  ascending  degeneration  of  these  tracts  so 
caused,  can  furnish  the  anatomical  substratum  for  combined  system  diseases. 
On  microscopical  examination  this  pathogenetic  subvariety  can  usually  be  ili^ 
tinguished  without  greal  difficulty  from  tin-  other  varieties  by  the  lack  of  sharp 
limitation  of  the  sclerosed  areas,  as  well  by  the  marked  vascular  alterations; 
Bymptomatologically,  however^the  result  can  he  identical.     Finally,  it  may  he 

emphasized  that  the  greater  susceptibility  of  the  pyramidal  tracts  and  the  pos- 
terior columns  to  ischemic  damage  makes  an  apparently  elective  affection  of 
just   these  tracts,  even   in  more  diffuse  vascular  lesions,  comprehensible.     In 

these  different  ways  the  combined  system  diseases  which  have  been  observed  in 
the  following  general  diseases  are  explainable:  Chronic  alcoholism,  chronic  lead 
poisoning,  diabetes,  carcinomatosis,  pellagra  (chronic  intoxication  by  spoiled 
corn),   lathyrism    (intoxication    from   chickpeas ) .   chronic    nephritis,    pernicious 

218 


214  LECTURE    XIV 

anemia,  leucemia,  sepsis.  In  practical  importance  these  conditions  are  far  in- 
ferior to  the  syphilogenic  combined  system  diseases  with  which  they  agree  en- 
tirely from  a  symptomatological  point  of  view.  The  description  of  these  last 
may  hence  serve  as  paradigm  for  the  whole  group. 

Let  us  add  that  in  the  production  of  syphilogenic  combined  system  diseases, 
in  part  simple  degenerative  processes  as  in  tabes,  in  part  tertiary  luetic  en- 
(darteritic  processes,  are  in  action.  The  disease  pictures  under  consideration 
are  hence  to  be  considered  partly  as  metasyphilitic,  partly  as  purely  syphilitic; 
their  description,  thus,  conducts  us  in  a  natural  manner  from  tabes  and  paresis 
to  cerebro-spinal  syphilis. 

The  combined  destruction  of  centripetal  systems  (affection  of  the  pos- 
terior column)  and  of  the  most  important  centrifugal  fiber  complexes  (Tractus 
cortico-spinalis)  has  as  a  consequence,  that  clinically  the  combination  of  atactic 
and  hypesthetic  phenomena  predominates.      Now,  however,   as  we  know  from 


- 

Fig.  67. 

Changes  in  the  Spinal  Cord  in  Syphilitic  Spinal  Paralysis.     Weipert-Pa!  Stain. 

our  former  descriptions,  elimination  of  the  pyramidal  tracts  has  as  its  conse- 
quence hypertonia  and  hyperreflexia,  that  of  the  posterior  fasciculi,  hypotonia 
and  hyporeflexia.  Hence,  in  combined  lesions  the  influence  of  diametrically 
opposed  tendencies  make  themselves  felt.  The  result  depends  upon  the  greater 
or  less  intensity  of  the  process  in  the  different  parts  affected. 

If  affection  of  the  pyramids  predominates  (see  Fig.  67),  there  results  the 
symptom-complex  of  spastic-atactic  paraplegia  with  sensory  disturbances  and 
we  call  such  cases,  after  Erb's  recommendation,  syphilitic  spinal  paralysis. 
The  sensory  disturbances  are  often  very  slight.  As  I  have  shown,  they  are,  as 
a  rule,  most  easily  recognizable  on  testing  vibration  sense  with  a  tuning-fork. 
Besides  this,  lancinating  pains,  bladder  disturbances,  reflex  pupillary  rigidity 
and  optic  atrophy  are  sometimes  observed,  just  as  in  tabes  dorsalis. 

If,  on  the  other  hand,  the  pyramidal  affection  is  less  than  that  of  the  sen- 
sory tracts  (see  Fig.  68)  a  syndrome  characterized  by  ataxia  and  arcflexia 
with  motor  weakness,  is  produced,  and  besides  this,  phenomena  pathognomonic 
for  the  pyramidal  affection  (chiefly  Babin&ki's  symptom,  more  rarely  that  of 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     215 

Oppenheim  or  of  Mendel-Bechterew)  usually  arc  present.  Also,  in  this  case, 
the  occurrence  of  lancinating  pains,  bladder  disturbances,  the  Argyll-Robertson 
pupil  and  optic  atrophy  are  not  rare,  and  we  speak  of  "combined  tabes." 

Pure  cases  of  syphilitic  spinal  paralysis  and  of  combined  tabes  are  rarer 
than  their  accompaniment  with  symptoms  that  belong  to  cerebro-spinal  syphilis. 

The  course  of  both  forms  is  slowly  progressive,  in  combined  tabes  on  the 
average  more  rapid  than  in  syphilitic  spinal  paralysis.  In  it  also,  the  prog- 
nosis is  clouded  by  the  not  so  rare  development  in  addition  of  general  paresis 
and  also  by  the  fact  that  specific  treatment  can  accomplish  much  less  than  in 
syphilitic  spinal  paralysis.  In  this  last,  indeed,  which  begins  often  in  the  first 
years    after   luetic    infection,    the    therapeutic    outlook    is    relatively   good.      If 


Fig.  <>8. 
The   Combined    Talus.      Weigert-Pal   Stain. 

the  treatment  is  begun  very  early  complete  recovery  is  possible;  if  later,  a 
definite  standstill  can  at  least  be  obtained,  and  to  this  the  disease  may  spon- 
taneously tend.  As  an  early  symptom  the  so-called  "spinal  intermittent  limp- 
ing" is  of  great  importance;  a  description  of  this  I  will  take  up  in  the  next 
lecture  when  speaking  of  arteriosclerotic  diseases  of  the  spinal  cord.  We  will 
consider   treatment   at    the  end   of  this   lecture. 


D.     Cerebro-Spinal  Syphilis 

In  the  great  majority  of  cases  in  which  tertiary  syphilitic  processes  de- 
velop in  tin-  central  nervous  system  they  appear  simultaneously  in  the  brain 
and  in  the  spinal  cord.  This  is  explained  by  the  facts  that  the  syphilitic  lesions 
of  the  nerve  centers  do  not  arise  from  the  parenchyma  proper,  the  ganglion 
cells  and  nerve  fibers,  but  are  partly  of  meningeal,  partly  of  vascular  origin, 


216  LECTURE    XIV 

and  that  both  the  meninges  and  the  vessels  of  the  brain  and  spinal  cord  present 
a  unique  and  connected  whole.  Let  us  enumerate  now  the  chief  alterations 
which  present  the  anatomical  substratum  of  cerebro-spinal  syphilis. 

1.  The  gumma  or  syphilitic  granulation  tumor  occurs  but  rarely  as  a  gross 
and  solitary  structure,  and  then  on  the  convexity  of  the  cerebrum;  its  place  of 
origin  is  then  usually  the  dura  mater  and  the  symptomatology  agrees  with 
that  of  true  neoplasms  of  corresponding  size  and  analogous  location.  Much 
more  frequent  is  the  formation  of  small  and  multiple  gummatous  nodules  which 
prefer  the  basal  portions  of  the  brain  and  usually  are  situated  in  the  pia- 
arachnoid  or  on  its  vessels.  Where  they  are  particularly  numerous  we  speak  of 
"gummatous  basilar  meningitis."  Also  the  gummata  developing  on  the  spinal 
cord  are  almost  always  multiple  and  originate  in  the  meninges ;  they  are  fre- 
quently miliary,  so  that  on  microscopical  examination  they  may  be  mistaken 
for  tuberculous  nodules. 

2.  Syphilitic  endarteritis,  a  disease  of  the  vessels  whose  chief  characteristic 
consists  in  a  progressive  thickening  of  the  intima  by  a  new  formed  connective 
tissue  rich  in  cells.  It  can  lead  to  complete  obliteration  of  the  lumen  of  the 
vessel.  As  a  rarity  the  deposit  of  minute  gummata  in  the  new  formed  endar- 
teritic  tissue  has  been  described.  Syphilitic  endarteritis  is  ubiquitous  in  the 
central  nervous  system;  it  can  be  found  as  well  in  the  meninges  or  on  the  sur- 
face of  the  brain  and  spinal  cord  as  in  their  interior. 

3.  Meningoencephalitis  and  meningo-mvelitis  syphilitica.  At  the  basis  of 
these  processes  lies  a  diffuse  infiltration  of  the  pia-arachnoid  with  small  cells, 
which  in  later  stages,  leads  to  considerable  connective  tissue  thickening  and 
infiltration  of  this  with  a  gelatinous  deposit.  Gummatous  deposits,  on  the 
other  hand,  are  absent  in  typical  cases.  In  the  altered  meningeal  region,  how- 
ever, the  vessels  without  exception  undergo  endarteritic  and  periarteritic 
changes ;  their  passing  into  the  superficial  portions  of  the  central  nervous 
system  furnishes  to  the  infiltration  process  a  port  of  entry  for  propagation 
in  the  cortex  of  the  brain  or  in  the  white  matter  of  the  spinal  cord.  In  the 
first  instance  the  convexity  of  the  hemispheres,  in  the  last,  the  posterior  part 
of  the  periphery  of  the  spinal  cord,  are  seats  of  £>redilection. 

Symptomatology 

a.  Cerebral  Symptoms. — A  particularly  constant  symptom  is  headache 
which  usually  has  a  paroxysmal  exacerbating  character  and  occurs  with  the 
greatest  severity,  particularly  at  night  or  early  in  the  morning.  At  the 
height  of  its  intensity  it  assumes,  usually,  a  boring  and  hammering  character; 
during  the  remissions  it  is  usually  described  as  a  dull  and  continuous  feeling 
of  pressure.  At  the  height  of  the  paroxysm  there  is  not  very  rarely  nausea 
or  vomiting,  on  which  account  on  superficial  diagnosis  it  is  occasionally  mis- 
taken for  migraine.  The  comparison  of  clinical  observations  with  autopsy 
findings  justifies  us  in  considering  this  headache  as  an  expression  of  basilar- 
meningitic  processes.  .Only  when  this  last  assumes  a  high  degree  of  intensity 
is  syphilitic  headache  accompanied  by  stiff  neck  and  rise  of  temperature. 
Circumscribed  sensitiveness  to  tapping  or  to  pressure  over  the  skull  is  scarcely 


SYPHILOGENTC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     217 

ever  to  be  found  in  syphilitic  basal  meningitis ;  where  it  is  found,  a  suspicion 
that  the  convexity  of  the  cerebrum  is  affected  is  justified. 

An  important  role  in  the  picture  of  cerebral  syphilis  is  played,  further, 
by  the  eye  symptoms,  which  often  involve  the  optic  nerve,  since  the  region 
of  the  chiasma  presents  a  point  of  predilection  for  the  localization  of  tertiary 
products.  Ophthalmoscopic  examination  shows  different  pictures,  among 
which  papilledema  and  optic  neuritis  depending  upon  increased  intracranial 
pressure  are  most  important.  We  will  consider  the  first  when  speaking  of 
brain  tumors  (Lecture  XIX)  ;  the  last — whose  end  result  is  neuritic  optic 
atrophy — has  already  been  considered  in  Lecture  II  among  diseases  of  the 
cranial  nerves.  Simple,  degenerative  optic  atrophy  is  sometimes  found  in 
cerebral  syphilis  and  in  a  form  entirely  agreeing  with  what  is  observed  in  tabes 
and  paresis;  its  occurrence  always  points  to  the  (quite  frequent  indeed)  com- 
bination of  syphilitic  and  mctasyphilitic  processes.  Optic  neuritis  can  occur 
unilaterally,  papilledema  and  degenerative  atrophy  are  practically  always 
bilateral,  indeed,  often  with  considerable  difference  in  intensity  between  right 
and  left.  Considerably  more  frequently  than  the  optic  nerve,  however,  the 
oculomotor  is  affected  and  indeed,  now  unilaterally,  now  bilaterally,  almost 
never  totally,  but,  as  a  rule,  with  avoidance  of  one  or  several  of  the  muscles 
supplied  by  it.  Particularly  regularly  and  particularly  early,  occurs  paralysis 
of  the  levator  palpebrae  muscle,  so  that  a  large  number  of  these  patients  are 
brought  to  the  physician,  usually  to  an  ophthalmologist,  on  account  of  ptosis 
as  the  first  symptom  of  their  brain  syphilis.  Rapid  change  in  the  picture  of 
the  eye  muscle  paralysis  (it  occurs  also  in  the  trochlear  and  the  abducens, 
though  considerably  less  frequently)  is  very  characteristic  of  brain  syphilis.'" 
As  to  syphilitic  affection  of  the  internal  branches  of  the  oculomotor,  paralysis 
of  the  pupils,  as  well  as  its  combination  with  paralysis  of  accommodation,  is 
found  (ophthalmoplegia  interna),  also,  finally,  the  reflex  pupillary  rigidity 
already  described  (Argyll-Robertson  pupil,  see  page  183).  The  pupillary* 
paralysis  naturally  manifests  itself  by  mydriasis  and  absence  of  reaction  of  the 
pupil  for  light  and  convergence. 

Of  other  luetic  cranial  nerve  symptoms  the  affections  of  the  trigeminus, 
facial,  auditory,  glosso-pharyngeal,  vagus,  and  hypoglossus  should  he  men- 
tioned. Tin1  trigeminus  manifests  its  involvement  in  the  disease  process  usually 
in  the  form  of  neuralgic  pains  (I  would  recall  to  you,  for  example,  the  bilateral 
Seeligmiiller's  neuralgia  of  the  auriculo-temporal  nerves  mentioned  in  Lecture 
II  (page  47),  as  characteristic  of  syphilis),  rarely  by  sensory  defect  symptoms 
or  loss  of  the  corneal  reflex,  only  very  exceptionally  in  the  form  of  trophic  dis- 
turbances, as,  for  example,  herpes  zoster,  corneal  ulcer,  etc.  On  the  part  of 
the  facial,  paralyses  of  peripheral  type  are  sometimes  observed  and  in  luetic 
basilar  meningitis,  indeed,  not  so  very  rarely,  as  the  otherwise  excessively  in- 
frequenl  facial  diplegia.  The  eighth  pair  of  nerves  are  very  frequently  affected, 
particularly  the  cochlear  (tinnitus  auriinn,  nerve  deafness)  here  and  there  also 
the  vest  il  nilar  branches  ( vevi  igo,  Meniere's  attacks)  ;  as  to  t  be  symptomatology, 
.i  page  •'!•'!  et  seq.  The  vagus  and  glosso-pharyngeal  roots  are  much  more 
rarely  affected,  and  then  almost  always  both  together;  their  involvemenl  mani 
fests  itself  in  attacks  of  cough,  difficulty  in  swallowing,  paralysis  of  I  be  vocal 


218  LECTURE    XIV 

cord,  the  (also  rare)  basal  syphilitic  lesion  of  the  hypoglossus,  in  hemi-atrophy 
of  the  tongue. 

In  the  vast  majority  of  cases  the  syphilitic  affections  of  the  cranial  nerves 
are  observed  not  isolated,  but  in  varied  combinations.  The  peculiarity  of  rapid 
change  in  the  symptom  picture  applies  not  only  to  the  syphilitic  eye  muscle 
paralyses  as  emphasized  above,  but  also  to  the  other  basal  phenomena. 

Brain  syphilis  can  further  lead  to  paralyses  in  the  extremities,  both  from 
basal  meningitis  and  particularly  from  endarteritic  changes.  This  occurs  almost 
always  as  one  of  the  varieties  of  hemiplegia.  We  will  postpone  the  considera- 
tion of  the  particularities  of  these  important  syndromes  to  a  future  lecture  in 
which  an  adequate  presentation  of  the  subject  of  half-sided  paralyses  of  cerebral 
origin  will  be  given  (Lecture  XVII). 

It  will  only  be  remarked  here  that  the  paralytic  symptoms  produced  by  the 
pressure  of  meningitic  exudates  upon  the  crus  cerebri,  the  pons,  and  the  medulla 
are  differentiated  by  their  less  intensity,  and  particularly  by  their  fugacity, 
from  the  prognostic-ally  decidedly  less  favorable  paralyses  of  vascular  origin. 
Endarteritic  processes  in  the  branches  of  the  basal  and  vertebral  arteries  can 
lead  to  bulbar  paralytic  disease  pictures  (see  Lecture  ATI,  page  120). 

As  to  gumma  and  to  gummatous  meningitis  of  the  cerebral  convexity,  with 
them  disease  processes  occur  which  greatly  resemble  those  which  we  will  study 
when  speaking  of  brain  tumors ;  indeed,  many  gummata  clinically  should  come 
under  the  classification  of  tumor.  For  syphilis  speaks  in  general  the  slight 
dev<  lopment  of  papilledema,  which  indeed,  in  gummata  of  considerable  size, 
is  sometimes  entirely  absent;  further,  a  striking  variation  in  the  intensity  of 
the  symptoms,  a  rapid  alternation  of  improvement  and  retrogression.  In 
general,  much  depends  upon  the  localization  of  the  syphilitic  process.  For  foci 
which  are  located  in  the  motor  area  of  the  cortex,  the  combination  of  the  so- 
called  "Jacksonian"  or  "cortical"  attacks  (see  Lecture  XIX)  with  relatively 
limited  unilateral  paralyses  (monoplegia  facialis,  faciobrachialis,  etc.)  is  typi- 
cal; left-sided  foci  often  lead  also  to  aphasic  disturbances  (see  Lecture  XVIII). 

Psychical  disturbances  point  nearly  always  to  diffuse  encephalitic  processes, 
particularly  to  extensive  disease  of  the  brain  arteries.  They  are  not  common 
in  gummata,  still  more  rare  in  syphilitic  meningitis  (apart  from  the  general 
clouding  of  the  sensorium  in  advanced  cases).  Upon  the  basis  of  diffuse  syphi- 
litic endarteritis,  disease  pictures,  which  may  deceptively  resemble  dementia 
paralytica  yet  are  distinguished  from  it  by  decidedly  better  prognosis,  often 
occur. 

As  chief  differential  diagnostic  criteria  for  distinguishing  them  from  true 
progressive  paralysis,  Kraepelin  and  Obersteiner  give  the  absence  of  general 
paretic  symptoms,  while  on  the  other  hand  focal  symptoms  may  occur  very  early 
and  are  much  more  obstinate,  the  absence,  or  much  less  marked  development  of 
disturbances  of  speech  and  handwriting ;  further,  disturbances  of  memory  and 
attention  keep  more  in  the  background,  reduction  of  sensitiveness  to  pain  is 
not  present ;  particularly  striking  is  the  great  frequency  of  hallucinations  of 
hearing.  Against  a  true  syphilitic  brain  disease,  but  only  with  caution,  a  great 
length  of  interval  between  infection  and  outbreak  of  the  disease  can  be  utilized. 
All  these  criteria  I  consider  of  very  questionable  value  and  I  could  refer  to 


SYPHILOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     219 

several  cases  in  which  by  psychiatric  authorities,  upon  the  basis  of  the  psychical 
syndrome,  a  luetic  psychosis  was  diagnosed  which  later,  however,  showed  them- 
selves to  be  true  paresis.  Wrong  diagnoses  can  never  be  entirely  avoided.  We 
are  most  justified  in  assuming  syphilitic  pseudo-paralysis  when  the  somatic 
symptoms  of  brain  syphilis,  particularly  the  cranial  nerve  paralyses,  are  very 
marked,  and  a  decided  alternation  of  symptoms  is  present,  mainly,  however, 
when  antisyphilitic  treatment  works  promptly  and  strikingly. 

b.  Spinal  Symptoms. — Syphilitic  meningomyelitis  and  spinal  endarteritis, 
the  syphilitic  granulomata  of  the  spinal  cord  and  its  membranes,  lead  to  clinical 
pictures  in  which  the  meningeal  and  the  medullary  components  of  the  anatomical 
substratum  find  expression  in  the  two  already  well  known  syndromes,  of  radicu- 
lar pains  and  partial  transverse  lesion ;  in  the  last  the  paralytic  symptoms 
appear  now  symmetrical,  that  is,  in  paraplegic  form,  again  unilateral  in  the 
form  of  the  Brown-Sequard  symptom  complex.  As  to  the  rest,  however,  the 
combination  of  flaccid  and  spastic,  sensory  and  motor,  irritative  and  defect 
phenomena,  varies  according  to  the  longitudinal  and  transverse  extent  of  the 
anatomical  lesions,  within  quite  wide  boundaries,  and  besides  this,  in  the  course 
of  the  disease  very  frequently  a  tendency  to  variation  in  intensity,  decided 
remissions  and  renewed  progressive  advances  are  noticeable. 

This  symptomatological  lability  can  render  a  differential  diagnosis  from 
multiple  sclerosis  considerably  more  difficult.  In  general  the  intensity  and  the 
frequency  of  bladder  troubles  is  much  greater  in  syphilitic  processes  than  in 
multiple  sclerosis.  This  applies  even  more  to  rectal  troubles  which  are  exces- 
sively rare  in  the  latter.  What  aid  we  are  justified  in  expecting  from  examina- 
tion of  the  spinal  fluid  in  cerebro-spinal  syphilis  has  been  already  thoroughly 
considered  in  Lecture  XII. 

That  the  spinal  symptoms  of  cerebro-spinal  syphilis  as  we  have  just  indi- 
cated them,  cannot  be  sharply  separated  from  those  of  the  "pseudo-systematic" 
forms  of  "syphilitic  spinal  paralysis"  should  be  clear  to  you  without  further 
remark;  also  as  compared  to  tabes  and  progressive  paralysis,  the  boundaries 
■in  frequently  bridged  over  by  the  combination  of  tertiary  and  metasyphilitic 
processes. 

Prognosis  and  Treatment 

The  prognosis  of  cerebro-spinal  syphilis  is,  presuming  timely  and  thorough 
specific  treatment,  decidedly  better  than  that  of  tabes  and  paresis,  indeed,  it 
BOH  he  said,  better  than  the  prognosis  of  the  other  organic  brain  and  spinal  cord 
affections.  The  more,  however,  the  cerebral  symptoms  occupy  the  foreground, 
the  more  serious  the  prognosis.  A  syphilis  located  principally  in  the  brain, 
sometimes  defies  the  most  energetic  therapy  and  leads  in  spite  of  all  our  efforts 
to  death  or  to  chronic  invalidism. 

Often  we  must  content  ourselves  with  incomplete  recovery,  or  "recovery 
with  defect."  The  best  outlook  is  given  by  the  spinal  syndrome,  where  some- 
times cures  bordering  on  the  marvelous  can  be  recorded.  I  have  already  men- 
tioned the  surprisingly  rapid  cure  of  a  complete  syphilitic  paraplegia  as  a 
result  of  treatment  by  salvarsan.  Energetic  mercurial  treatment  can,  though 
■MB    rapid,   effeci    just    as   striking   results    in    cerebro-spinal    syphilis.      With 


220  LECTURE    XIV 

all  recognition  of  the  powerful  agent  which  we  have  in  salvarsan,  this  must 
be  expressly  emphasized,  as  Oppcnhtim,  Nonne,  Sanger  and  others  have  done. 
Personally,  my  position  is  that  in  great  development  of  cerebral  symptoms  I 
still  hesitate  to  recommend  its  use  instead  of  that  of  mercury,  since  the  aggra- 
vation of  the  symptoms  as  they  have  been  observed  in  such  cases,  after  the 
use  of  salvarsan  by  Mlngazzini  and  others  (the  risk  of  them  is  small,  it  is  true) 
need  not  be  feared  in  the  employment  of  the  less  heroic  but  nevertheless  actively 
efficient  mercurial  treatment.  Where,  however,  salvarsan  is  to  be  given  (often 
in  response  to  the  direct  wish  of  the  patient,  in  which  event  it  is  advisable  after 
Oppenheim's  recommendation,  to  insist  that  he  assume  responsibility  for  any 
untoward  results  before  beginning  the  treatment),  it  should  not  be  used  in 
insufficient  doses,  since  the  "neurcrezidive"  observed  after  arsenobenzol  injec- 
tions (they  are  usually  paralyses  of  the  cranial  nerves)  are  probably  not  due 
to  direct  toxic  action  of  the  remedy  but  are  of  true  syphilitic  nature  and  their 
occurrence  is  favored  by  the  "neurotropy"  of  the  salvarsan,  but  only  with  the 
provision  that  the  spirocha'tae  after  the  injection  still  have  sufficient  power 
to  cause  organic  lesions.  A  good  rule  for  salvarsan  treatment  is  presented  in 
the  scheme  of  Bruno  Block:  a.  Intravenous  salvarsan  injection  (0.6  grm  in 
adults.  In  delicate  persons,  in  children,  etc.,  correspondingly  less),  b.  5  to 
7  days  later  the  first  intramuscular  salvarsan  injection  of  0.3  grm.  c.  14  to 
20  days  after  the  intravenous  injection  a  seconr  intramuscular  injection  of 
0.3  grm.  d.  4  to  5  weeks  after  the  first  intravenc  5  injection,  finally,  a  second 
intravenous  injection. 

With  regard  to  the  use  of  mercury  many  neurologists  still  give  preference 
to  the  old  inunction  treatment.  However  true  it  is  that  this  latter  properly 
carried  out  and  sufficiently  frequently  repeated  at  graduated  intervals  can 
accomplish  most  excellent  results,  it  is  certain  that  exactly  the  same  thing 
can  be  accomplished  with  the  much  more  convenient  and  cleanly  intramuscular 
injections,  on  which  account  I  use  these  almost  exclusively.  Where  there  is 
danger  in  delay  and  very  rapid  mercurialization  is  necessary  I  usually  begin 
with  daily  injections  of  a  soluble  salt  of  mercury,  for  example,  the  biniodide 
according  to  the  formula  given  on  page  210;  instead  of  the  daily  dose  of  0.02, 
larger  doses  (0.03  to  0.04)  can  be  given,  indeed,  with  risk  of  acute  mercurial 
intoxication.  This  we  can  arrest,  however,  when  using  the  soluble  salts  of 
mercury,  by  stopping  the  injections,  which  we  cannot  do  with  the  still  more 
active  but  insoluble  calomel  injections  (in  40  per  cent,  emulsion)  on  which 
account  this  last  method  is  best  left  to  the  experienced  syphilologist.  After  10 
days  the  injections  of  biniodide  of  mercury  are  intermitted  for  several  days. 
A  series  of  20  to  30  injections  constitute  a  "cure." 

In  later  treatment  under  less  pressing  indications,  injections  of  salicylate 
or  thymoloacctate  of  mercury  (in  10  per  cent,  emulsion  with  paraffine  oil) 
should  be  preferred  to  the  soluble  salts,  since  on  account  of  its  more  protracted 
absorption  the  injections  are  at  longer  intervals,  hence  more  convenient  and 
less  expensive.  Twelve  injections  of  1  cc  each  at  intervals  of  from  3  to  4 
days  constitute  a  normal  "cure"  for  a  strong  adult ;  it  is  well,  however,  to  test 
the  tolerance  at  first  by  giving  smaller  doses.  That  in  all  mercurial  injections 
the  most  careful  asepsis  and  proper  technique  is  necessary  goes  without  saying. 


SYPHIEOGENIC  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     221 

Place  of  injection:  External  upper  quadrant  of  the  gluteal  region;  with 
avoidance  of  vessels  and  regular  examination  of  the  urine  for  albumin  should 
never  be  neglected,  particularly  when  large  doses  are  used. 

The  erroneous  idea  that  cerebro-spinal  syphilis,  since  it  is  "tertiary,"  is  to 
be  cured  with  iodide  of  potassium  alone,  must  be  decidedly  opposed.  Iodide 
of  potassium,  however,  is  to  be  used  and  in  not  too  small  doses  along  with 
mercury  and  eventually,  salvarsan.  I  increase  it  usually  to  i  to  6  grms  a  day ; 
many  syphilologists  go  considerabl}'  higher  (to  15  grm).  After  each  20 
days  it  is  intermitted  for  about  10  days.  If  iodism  begins,  a  solution  of  3  grms 
each  of  sulphanilic  acid  and  bicarbonate  of  soda  in  200  cc  water  is  given.  As 
substitutes  for  iodide  of  potassium  (over  which  iodide  of  sodium  has  no  advan- 
tages) when  there  are  symptoms  of  intolerance,  different  organic  iodine  prep- 
arations come  under  consideration,  for  example,  sajodin,  iodgline,  iodtropon, 
iodstarin,  lipoiodin,  etc.  They  are  all,  however,  less  active  with  the  exception 
of  intramuscular  injections  of  from  10  to  20  cc  of  25  per  cent,  sajodin,  which 
repeated  at  intervals  of  from  3  to  4  days  sometimes  has  striking  effect. 

Internal  administration  of  mercury  is  at  most  to  be  made  use  of  for  later 
mild  "intercures" ;  under  no  circumstances  can  it  replace  injection  or  inunction 
cures.  I  would  mention  in  this  connection  mercurous  tannate  0.05  (gr.  %) 
in  pill  form  t.i.d. 

In  conclusion  a  word  8  to  the  relations  between  the  Wassermann  reaction 
and  the  treatment  of  cerebi  rspinal  syphilis;  it  occurs  not  infrequently  that  in 
cases  where  this  reaction  is  positive,  even  the  most  energetic  specific  treatment 
cannot  change  it  into  negative.  This,  however,  in  no  way  clouds  the  prognosis, 
since  in  spite  of  this,  recovery  can  be  obtained  and  preserved.  In  order  that 
such  patients  do  not  become  unhappy  syphilophobes,  we  must  practice  a  "pious 
fraud"  and  not  inform  them  of  the  continued  anomaly  of  their  blood  serum, 
lest  they  believe  themselves  continually  threatened  with  a  recurrence  and  doomed 
to  life-long  use  of  mercury. 


LECTURE    XV 

Arteriosclerosis  of  the  Nerve  Centers 

Gentlemen  :  In  to-day's  lecture  we  will  consider  neither  the  mechanical 
destruction  which  the  nervous  tissue  suffers  in  consequence  of  the  rupture  of 
a  vessel  altered  by  arteriosclerosis,  nor  the  extended  foci  of  softening  which 
the  atheromatous  closure  of  a  large  artery  produces. 

These  gross  and  striking  results  of  arteriosclerosis  of  the  central  nervous 
system  will  be  described  at  length  in  a  later  lecture. 

To-day  I  will  take  up  those  phenomena  which  are  explainable  on  the  ground 
of  diffuse  disturbances  of  nutrition  in  the  brain  and  spinal  cord,  consequent 
upon  alteration  of  their  vessels,  as  well  as  certain  paroxysmal  or  intermittent 
nervous  disturbances,  whose  comparison  with  other  arteriosclerotic  symptom 
complexes  (for  example,  angina  pectoris  or  intermittent  limping)  imposes  itself 
upon  us.  Further,  I  will  unfold  before  you.  different  clinical  pictures  which, 
in  spite  of  their  relative  frequency,  are  still  too  little  considered  and  which 
stand  in  relationship  with  special  peculiar  modalities  of  arteriosclerotic  tissue 
destruction. 

Symptomatology 

Of  special  practical  importance  is  acquaintance  with  that  category  of  dis- 
ease manifestations  which  we  can  unite  under  the  name  of  arteriosclerotic 
pseudo-neurasthenia.  In  the  proper  estimation  and  management  of  just  this 
condition,  of  which  Windscheid,  Erlenmeyer  and  others  have  given  us  good 
descriptions,  we  will  find  the  central  point  of  prophylaxis  against  all  destruc- 
tive nerve  lesions  of  arteriosclerotic  origin,  for  example,  also  against  the  cere- 
bral hemorrhages  and  foci  of  softening  already  mentioned.  It  is  plainly  the 
very  first  beginning  of  vascular  degeneration  in  the  central  nervous  system. 

The  patients — usually  men  between  40  and  55  years  old — generally  come 
under  treatment  on  account  of  headache.  This  is  usually  a  feeling  of  pres- 
sure and  heaviness,  particularly  about  the  forehead  which  is  present  on  rising 
and  increases  during  the  day  to  a  considerable,  often  to  a  distressing,  intensity. 
Often  the  headache  increases  particularly  on  mental  effort,  sometimes  even  on 
meditation,  which  the  French  author  Josne  characterizes  as  "le  signe  de  la 
pensee  douloureuse"  (symptom  of  painful  thinking).  There  are  also  cases 
in  which  headache  is  absent  during  complete  rest  but  is  produced  by  the  slight- 
est mental  or  bodily  effort.  This  headache  can  present  a  great  similarity  to 
that  produced  by  anomalies  of  refraction,  or  by  moderate  astigmatism  in  people 
beyond  40  years  old — when  the  power  of  accommodation  decreases.  It  will 
be  well  in  such  patients  not  to  neglect  the  eye  condition. 

Further,  there   is   frequent  complaint   of  vertigo;  sometimes   this  designa- 

222 


ARTERIOSCLEROSIS    OF    THE    NERVE    CENTERS  223 

tion  is  applicable  (namely  for  sensations  which  the  patients  experience  in  the 
morning  upon  sitting  up  in  bed  or  upon  rising)  ;  often,  however,  under  this 
so  frequently  misused  expression  are  understood  only  sudden  feelings  of  weak- 
ness or  momentary  darkening  of  the  visual  field,  or  finally,  feelings  of  oppres- 
sion or  anxiety.  Only  rarely  do  we  encounter  true  rotary  vertigo  to  the  ves- 
tibular nature  of  which  an  accompanying  tinnitus  can  point,  although  the 
otological  examination  may  disclose  no  middle  ear  or  labyrinthine  affection.  A 
noteworthy  variety  of  arteriosclerotic  vertigo  which  as  far  as  I  know  was  first 
described  by  Homburger,  I  have  heard  occasionally  sketched;  when  the  patient 
lies  in  bed  he  suffers  from  the  painful  uncertainty  of  not  knowing  exactly  how 
he  is  lying — whether  upon  his  back  or  upon  his  side — and  cannot  describe  the 
location  of  his  bed;  with  this  a  distressing  anxiety  and  oppression  may  come 
over  him  and  may  be  accompanied  with  a  sensation  of  a  dull  humming  inside  of 
his  skull.  Almost  always,  also,  there  are  sleep  disturbances,  which,  however, 
scarcely  ever  lead  to  a  severe  and  obstinate  agrypnia,  as  for  example,  in  the 
prodromal  stage  of  general  paresis.  Usually  it  is  only  considerable  difficulty 
in  going  to  sleep,  restless  sleep  and  early  awakening  with  a  sense  of  weight  in 
the  head.  Rather  rare  and  somewhat  limited  to  those  of  more  advanced  age 
among  the  arteriosclerotics,  is  the  combination  of  ability  to  sleep  well  by  day 
with  nocturnal  sleeplessness  and  jactitation.  This  paradoxical  phenomenon 
has  been  called  "sleep  inversion."  You  will  often  note,  also,  that  in  arterio- 
sclerotics the  intensity  of  the  sleep  disturbances  varies  from  day  to  day. 

Ability  to  work  suffers  usually  in  this  manner,  that  the  patient  can  follow 
his  ordinary  occupation,  but  contrary  to  what  was  formerly  the  case,  finds 
it  a  great  exertion  and  is  no  longer  able  to  take  up  new  tasks.  Mental  con- 
centration  is  particularly  difficult,  which  is  brought  to  the  realization  of  the 
patient  on  reading  a  paper,  in  keeping  his  accounts,  in  hearing  a  lecture  or 
sermon,  etc. 

The  mood  is  usually  fretful,  irritable,  morose,  and  besides  this  there  often 
makes  itself  felt  a  pessimism  due  to  the  feeling  of  reduced  capacity  which  first 
concerns  the  health  and  economic  future  of  the  patient,  but  soon,  however, 
disturbs   his   judgment   of  all   relationships. 

Finally,  many  of  these  patients  complain  of  abnormal  sensations,  usually 
in  the  form  of  formication,  prickling,  going  to  sleep  of  the  extremities. 

These  troubles  present  a  great  similarity  to  neurasthenic  symptom-com- 
plexes (see  below.  Lecture  XXVII).  The  differential  diagnosis  from  neuras- 
thenia is  sometimes  made  easier  for  us  by  going  thoroughly  into  the  history. 
No  neurasthenia  occurs  without  some  definite  etiological  preceding  conditions. 
In  the  constitutional  form  of  this  disease  its  congenital  degenerative  basis  is 
clear  from  the  fad  that  its  first  beginnings  can  be  traced  back  into  early 
youth;  in  the  acquired,  accidental  form,  on  the  other  hand,  «c  can  regularly 
make  definite  exogenic  causal  factors  responsible,  among  which  there  is  usually 
a  combination  of  nervous  fatigue  in  any  direction  with  the  chronic  action  of 
disagreeable  emotions.  A  nervosity,  however,  which  begins  apparently  without 
cause  in  a  person  of  "arteriosclerotic  age"  of  previously  healthy  nervous  sys- 
tem, imperatively  demands  the  close  investigation  of  the  vascular  apparatus. 
And  even  in  genuine  inveterate  neurasthenics  it  Ihis  critical  ace,  arteriosclerosis 


224  LECTURE    XV 

should  conscientiously  be  sought  for ;  it  is  indeed,  a  fact  long  known,  that 
neurasthenics — mainly  those  with  vasomotor  cardiac  symptoms — are  predis- 
posed to  arteriosclerosis.  Mutual  relations  between  both  disease  conditions 
has,  a  priori,  much  probability.  I  have  pointed  out  that  cases  of  neurasthenia 
with  cardiac  and  vascular  symptoms  ( for  example,  tachycardia,  dermograph- 
ism, etc.)  are  characterized  very  often  by  abnormally  high  blood  pressure  (see 
Lecture  XXVII).  Now,  however,  cardio-vascular  hypertension,  as  has  been 
experimentally  recognized,  plays  a  role  in  favoring  the  occurrence  of  atheroma- 
tous heart  lesions. 

I  cannot  refrain  from  mentioning  at  this  opportunity  the  recognition  by 
the  American  physiologist  Cannon  that  in  animals  psychical  excitement  (in 
the  cat,  for  example,  coming  into  contact  with  a  dog)  produces  an  adrenal- 
inema.  This  fact  appears  to  me  to  throw  a  new  light  upon  the  pathogenetic 
role  which  in  the  etiology  of  arteriosclerosis  may  be  attributed  to  the  con- 
tinuous increased  emotivity  common  to  all  neurasthenics.  Now  adrenaline 
acts  injuriously  upon  the  vessel  walls,  both  chemically  and  by  raising  the  blood 
pressure.  Finally,  a  still  more  direct  connection  between  the  nervous  system 
and  vascular  degeneration  has  been  experimentally  rendered  probable ;  for  ex- 
ample, Manouclian  has  shown,  that  through  the  destruction  of  definite  nerve 
twigs  entering  the  vessel  walls,  sclerotic  plaques  may  be  produced  in  a  corre- 
sponding situation. 

To  return  to  the  clinical  side,  if  there  is  a  suspicion  of  arteriosclerotic  ner- 
vosity in  a  patient,  we  must  naturally  confirm  the  diagnosis  by  recognizing 
the  vascular  affection.  Here  I  would  warn  you  above  everything  against 
depending  upon  the  palpation  of  the  peripheral  arteries  as  is  so  often  done. 
The  clinic  and  the  autopsy  table  in  agreement,  teach  us  that  between  the 
condition  of  the  peripheral  arteries  and  that  of  the  brain  and  spinal  cord 
vessels,  a  paradoxical  incongruence  often  exists.  I  could  mention  many  cases 
of  cerebral  hemorrhage  and  encephalomalacia  with  severe  sclerotic  alterations 
of  the  brain  arteries,  in  which  in  the  radial,  the  dorsalis  pedis,  the  carotid,  the 
brachial,  and  even  the  temporal,  there  was  no  perceptible  tortuosity  or  harden- 
ing of  the  vessels.  Nevertheless,  I  would  add  that  (when  all  arteries  accessible 
to  palpation  are  examined)  negative  findings  in  this  respect  are  not  so  very 
frequent. 

As  to  the  rest,  in  arteriosclerotic  pseudo-neurasthenia  we  can  pretty  well 
count  on  registering  further  stigmata  of  vascular  disease  in  these  patients, 
sometimes  indeed,  in  considerable  number.  So,  for  example,  alteration  of  the 
size  of  the  heart,  especially  hypertrophy  of  the  left  ventricle,  accentuation  of 
the  second,  impurity  of  the  first  aortic  sound. 

Slight  albuminuria  is  usually  to  be  found  if  the  urine  is  repeatedly  examined ; 
and  also  in  urine  free  from  albumin,  hyaline  and  granular  casts  are  occasionally 
observed.  Of  importance,  also,  is  the  recognition  of  slight  oedema  over  the 
ankles  in  the  evening,  provided  that  it  is  not  explainable  through  heart  weak- 
ness, nephritis,  or  varicose  veins.  Sometimes  there  is  a  tendency  to  nose  bleed. 
As  to  stenocardiac  attacks  proper,  or  cardiac  asthma,  it  is,  as  a  rule,  not 
necessary  to  inquire,  as  these  conditions  are  usually  spontaneously  mentioned 
by   the   patient ;   still   they   occur   sometimes   in   slight    or   rudimentary   form, 


ARTERIOSCLEROSIS    OF   THE    NERVE    CENTERS  225 

scarcely  observed  by  him;  this  applies,  namely,  to  the  "respiratory  fatigue"' 
of  the  French,  which  occurs  on  moderate  bodily  exertion.  It  is  well,  also,  to 
measure  the  blood  pressure,  although  its  increase  which  according  to  Broedel 
occurs  in  about  65  per  cent,  of  cases  of  Arteriosclerosis,  according  to  Romberg 
and  others,  is  no  symptom  of  the  vascular  disease  itself,  but  of  the  interstitial 
nephritis  which  so  often  accompanies  this  last. 

Of  late  it  has  been  endeavored  by  Kurt  Mendel,  in  disease  pictures  which 
occur  without  any  external  cause,  in  previously  healthy  men  between  45  and 
50  years  of  age  and  which  closely  correspond  to  the  description  which  I  have 
given  you  of  arteriosclerotic  pseudo-neurasthenia,  in  which,  however  (if  we 
neglect  congestions  of  the  head  and  paroxysmal  palpitation),  cardiac  vascular 
anomalies  are  absent,  to  refer  these  cases  as  "molimina  climacteria  virilia"  to 
alterations  of  the  inner  secretion  of  the  genital  glands  and  to  separate  them 
from  incipient  arteriosclerosis  of  the  nerve  centers. 

We  will  turn  now  to  a  group  of  disturbances  of  considerable  intensity  but 
of  fleeting  character  which  we  sometimes  see  appear  upon  the  basis  of  arterio- 
sclerotic pseudo-neurasthenia  and  which  demand  our  close  attention.  They 
inform  us,  namely,  of  an  already  high  degree  of  intensity  of  the  vascular  dis- 
ease and  represent  only  too  often  (as  the  further  course  of  such  cases  teaches 
us)  the  forerunners  of  irreparable  organic  lesions  of  the  nervous  system. 

I  would  mention  first,  severe  pains  which  occur  in  attacks,  are  characterized 
by  great  intensity,  and  are  located  both  on  the  trunk,  in  the  limbs  and  in  the 
region  of  the  trigeminus.  They  are  mainly  considered  as  neuralgias,  although 
I  believe,  incorrectly  very  often.  The  absence  of  pressure  points  along  the 
nerves  in  question,  the  boring,  lancinating,  constricting  character  of  these 
attacks,  recalling  tabes  dorsalis,  makes  it  appear  probable  to  me  that  they 
are  due  to  vascular  spasms  in  the  central  nervous  organs  or  in  their  posterior 
roots.  Nothnagel  and  Huchard  long  ago  emphasized  the  role  in  the  clinical 
picture  of  arteriosclerosis,  which  vascular  spasm  occurring  in  attacks,  played 
alongside  of  the  permanent  vascular  lesions,  and  the  similarity  of  these  painful 
crises  of  arteriosclerotics  to  angina  pectoris  and  to  arteriosclerotic  colic,  de- 
cidedly impresses  itself.  Still  further  analogies,  however,  exist  between  certain 
intermittent  disturbances  in  the  motor  functions,  which  are  occasionally  found 
in  cerebro-spinal  arteriosclerosis  and  the  interesting  phenomenon  which  is  known 
as  "intermittent    limping." 

This  complaint  ("Claudicatio  intermittens,"  "Dysbasia  angiosclerotic  in- 
termittens") often  presents  a  prodromal  stage  of  the  so-called  spontaneous 
gangrene  of  the  lower  extremities.  It  is  caused  by  arteriosclerosis  of  the  leg 
arteries,  though  functional  causes  also  play  a  role.  While  namely,  on  walk- 
ing, the  movements  of  such  patients  are  first  normal,  painless  and  unhindered, 
after  :i  certain  time — this  can  be  in  one  case,  a  few  minutes,  in  another 
a  half  or  even  three-quarters  of  an  hour — there  occurs  an  extreme  weak- 
ness of  the  lower  extremities  with  cramplike  sensation,  which  soon  makes 
further  progression  impossible  f<>r  the  patient.  At  the  same  time  it  can  be 
remarked  how  single  toes,  single  portions  of  the  skin  of  the  feet,  alter  their 
color  under  the  eyes  of  the  observer,  now  deathly  pale,  now  livid,  again  cyanotic 
— a  plain  evidence   that    it   is   not    exclusively  the  expression  of  blood   supply 


226  LECTURE    XV 

insufficient  for  the  locomotor  requirements,  but  that  a  vasomotor  spasm  is  also 
in  action — after  a  short  rest,  however,  all  the  trouble  has  disappeared  again 
and  the  patient  can  move  in  a  normal  manner ;  indeed,  only  after  about  the 
same  time  as  formerly,  to  be  again  attacked  by  the  trouble  and  to  be  compelled 
to  rest.  And  so  the  same  thing  is  repeated  upon  all  attempts  at  walking; 
with  the  total  duration  of  the  locomotor  effort,  the  time  of  unhindered  walk- 
ing usually  becoming  continually  shorter.  Dysbasia  angiosclerotica  inter- 
mittens, occurs  very  rarely  with  us ;  in  Poland  and  Russia,  however,  it  is  strik- 
ingly frequent,  which  may  have  a  connection  with  the  very  cold  climate.  There 
is  in  that  region  an  apparent  predisposition  among  the  Jewish  race  which  is 
not  the  case  in  our  neighborhood  ( of  5  cases  which  I  have  seen,  not  one  was  a 
Jew).  Excessive  smoking  certainly  plays  an  important  etiological  role.  The 
treatment — apart  from  the  general  treatment  of  arteriosclerosis — falls  in  with 
that  of  the  vasomotor  neurotic  forms  of  intermittent  limping  (see  Lecture 
XXV). 

There  occurs  now  in  arteriosclerosis  of  the  central  nervous  system  a  dis- 
turbance which  appears  so  like  the  classical  picture  of  intermittent  claudication 
introduced  into  the  nosology  by  Charcot  and  Erb,  that  Dcjerine  denominates 
it  as  the  spinal  variety  of  this  disease,  an  "intermittent  claudication  of  the 
spinal  cord."  I  have  seen  two  exceedingly  instructive  cases  of  this.  In  dis- 
tinction from  the  peripheral  form,  all  vasomotor  disturbance  in  the  lower 
extremities  is  absent  and  both  arteries  on  the  feet  (dorsalis  pedis  and  tibialis 
posticus)  are  to  be  felt  normal.  With  the  beginning  of  the  limping,  there 
occur  the  following  symptoms  characteristic  of  it:  an  increase  in  the  patellar 
and  Achilles  reflexes,  often  a  decided  ankle  clonus,  and  sometimes  also,  the 
Babinsky  reflex,  as  clinical  corollaries  of  an  ischemic  condition  of  the  pyramidal 
tracts.  Somewhat  more  frequently  than  in  arteriosclerosis  of  the  spinal  cord 
— it  can  be  said  in  passing — spinal  intermittent  limping  has  been  observed  upon 
a  basis  of  syphilitic  endarteritis. 

Along  with  the  just  described  paretic  paroxysms  of  spinal  origin  we  can 
observe  those  of  plainly  cerebral  origin,  although  much  less  frequently,  in 
which,  however,  the  cooperation  of  muscular  effort  as  an  exciting  cause  is  less 
plain,  or  indeed,  cannot  be  recognized.  I  have  in  mind  particularly  the  tem- 
porary paresis  of  an  arm  or  leg  or  even  of  both  extremities  on  one  side  of  the 
body  which  usually  greatly  frighten  the  patient,  but  after  a  few  hours,  or 
even  minutes,  entirely  disappear.  Still  more  interesting  is  the  temporary  motor 
aphasia,  of  which  I  might  mention  three  observations:  in  one,  the  disturbance 
lasted  only  a  few  minutes,  in  the  others,  from  1.5  to  20  minutes.  One  of  these 
last  cases  seemed  to  illustrate  the  influence  of  functional  use,  in  that  the  woman 
affected  afterward  explained  that  on  the  day  on  which  it  occurred  she  hail  felt 
very  much  fatigued  from  long  talking  to  visitors. 

All  these  transitory  phenomena,  however  striking  their  method  of  occur- 
rence may  be,  always  stand  on  the  border  between  the  functional  and  the 
organic.  They  can  be  considered  as  the  original  expression  of  Grassefs  has 
it,  "avertissenients  sans  frais"  (free  warnings).  The  disturbances,  however,  to 
which  we  will  now  turn,  depend  upon  material  alterations  which,  although  not 
very  extensive,  are  nevertheless  of  far-reaching  importance. 


ARTERIOSCLEROSIS   OF   THE    NERVE    CENTERS  227 

I  cannot  go  any  farther  into  arteriosclerotic  diseases  of  the  spinal  cord 
(as  a  forerunner  of  which  we  have  become  acquainted  with  spinal  intermittent 
claudication)  but  will  only  state  that  a  degeneration  in  the  region  of  the 
lateral  columns  due  to  arterial  ischemia'  in  consequence  of  atheromatous  oblit- 
eration of  vessels  can  produce  the  symptom-complex  of  spastic  spinal  paralysis. 
These  cases  which  have  been  isolated  by  Dcmange,  Oppenheim  and  others  as 
senile  spastic  paraplegia,  affect  usually  only  the  lower  extremities,  very  rarely 
the  arms  also.  A  more  extended  affection  of  the  vascular  apparatus  of  the 
.-pinal  cord  can  also  produce  sensory  disturbances  and  sphincter  paralysis. 

These  findings  are  much  less  frequent  than  the  extensive  symptoms  which 
small,  unapparent  losses  of  substance  due  to  arteriosclerosis  in  the  brain  can 
produce,  thanks  to  the  multiplicity  which,  as  a  rule,  characterizes  their  occur- 
rence. These  are  the  so-called  "Lacuna1"  ("lacunes  de  desintegration  cere- 
brate," "etat  lacunaire  du  cerveau")  first  thoroughly  studied  by  Pierre  Metric 
in  Paris.  From  miliary,  to  the  size  of  a  pea,  of  irregular  outline,  these  losses 
of  substance  pervade  in  greater  or  less  number  circumscribed  areas  of  the 
cerebrum,  the  interbrain,  ami  the  midbrain.  In  the  middle  of  each  one  of 
these  small  spaces  located  close  together,  we  notice  upon  microscopical  exam- 
ination an  arteriosclerotic  but  still  pervious  vessel;  they  cannot  hence  be  con- 
sidered as  small  areas  of  softening  due  to  the  closure  of  vessels.  Rather  have 
the  newest  histological  investigations  shown  that  inflammatory  processes  of 
the  adventitia  and  of  the  peri-arterial  lymph  spaces  are  responsible  for  the 
destruction  of  the  surrounding  brain  tissue. 

The  status  lacunaris  of  the  brain  is  properly  to  be  distinguished  from  the 
so-called  "status  cribrosus"  of  Durand-Fardel,  which  results  from  dilatations 
of  the  peri-vascular  lymph  sheaths,  while  it  is  pathogcnieally  more  nearly  re- 
lated to  a  lesion  of  the  cerebral  cortex  described  by  Marie,  Alzheimer  and 
others,  which  gives  to  this  last  a  worm-eaten  appearance  ("etat  vermoulu") 
since  it  is  dui'  to  the  destruction  of  cortical  substance  about  diseased  arteries. 
On  the  other  band,  the  "cerebral  porosis"  of  certain  pathological  anatomists 
(also  called  "cystic  degeneration"  or  "'etat  de  fromage  de  Gruyere")  is  nothing 
else  but  a  post-mortem  artefact  produced  by  the  bacillus  serogenes  capsulatus, 
by  tin'  aid  of  which  these  changes  can  be  experimentally  produced  also. 

A  special  variety  of  I  be  hemiplegia  of  old  age  depends,  too,  upon  arterio- 
sclerotic formation  of  lacuna'.  This  lacunar  hemiplegia  (to  anticipate  our 
later  remarks,  Lecture  XVII)  can  usually  be  distinguished  without  difficulty 
from  the  hemorrhagic,  thrombotic  and  embolic  hemiplegias  by  a  number  of 
clinical  criteria.  These  criteria  are  particularly  in  tin'  character  and  course 
of  the  attack,  which  almost  never  presents  the  picture  of  a  true  apoplectic 
seizure,  namely,  it'  it  does  not  occur  at  nighl  (which  it  often  does)  it  usually 
runs  its  course  with  retained  consciousness  and  at  most,  some  vertigo  and  a 
temporary  confusion  are  its  accompaniments.  In  the  rare  cases,  however,  ill 
which  the  hemiplegic  paralysis  benins  violently  (this  is  the  case  when  the 
lacuna'  involve  the  internal  capsule)  the  loss  of  consciousness  is  of  very  short 
duration— lasts  less  than  an  hour.  The  resulting  hemiplegia  is.  further,  usually 
of  little  intensity  and  markedly  transitory  character.  Although  it  also  leads 
to  exaggeration  of  reflexes,   it   never   causes  contractures,  but   more  or   less 


228  LECTURE    XV 

completely  disappears  after  hours,  clays  or  weeks.  (A  longer  duration  of  the 
hemiplegic  symptoms  again  points  to  the  seat  in  the  capsule  of  the  tissue  de- 
fects.) The  lower  extremities  are  usually  longest  affected,  since  a  certain 
regularity  in  the  distribution  of  the  paralytic  symptoms  is  not  to  be  mistaken. 
Among  these,  most  important  is  the  absence  of  aphasic  symptoms,  while  there 
may  be  some  disarthria;   further,  we  miss   regularly  hemianopsia,  conjugate 


Status  Laeunaris  Cerebri.     Typical  attitude  and  "demarche  a.  petit  pas." 

deviation,  and  appreciable  disturbances  of  sensibility,  while  quite  frequently 
the  facial  nerve  is  unaffected.  Ankle  clonus  and  the  Babmsky  and  Oppcnlieim 
phenomena  can  often  be  observed ;  on  the  other  hand  the  abdominal  reflexes 
are  scarcely  ever  disturbed.  Now,  however,  after  the  hemiplegic  symptoms 
have  disappeared  with  remarkable  rapidity  and  completeness,  a  change  which 
gives  to  such  patients  a  quite  characteristic  attitude  progressively  sets  in. 
While,  in  spite  of  persistent  anomalies  of  reflexes,  the  paretic  symptoms  at 


ARTERIOSCLEROSIS    OF   THE    NERVE    CENTERS  229 

most  manifest  themselves  in  the  fine  movements,  for  example,  in  buttoning  the 
clothes,  the  gait  takes  on  an  exceedingly  typical  character  (Fig.  69).  In  the 
yards  and  gardens  of  asylums  for  old  people  and  almshouses,  such  patients  can 
be  seen  going  about,  their  bodies  bent  forward,  the  knees  in  half-flexion,  not 
spastic,  indeed,  but  with  inflexible  joints,  and  slowly  dragging  one  foot  after 
the  other,  the  soles  scraping  the  ground.  A  certain  similarity  between  this 
"demarche  a  petit  pas"  (gait  of  little  steps)  as  Brissaud  has  called  it,  and  the 
gait  of  patients  with  paralysis  agitans  (see  Lecture  V)  is  not  to  be  denied, 
though  the  differential  diagnosis  presents  no  difficulty  to  those  familiar  with 
both  conditions. 

We  leave  now  the  lacunar  hemiplegias  to  turn  to  a  disease  picture  which 
in  a  large  percentage  of  cases  also  depends  upon  arteriosclerotic  tissue  defects, 
sometimes,  indeed,  from  disseminated  arteriosclerotic  foci  of  another  character, 
for  example,  miliary  hemorrhages  or  fibro-hyaline  obliteration  of  vessels.  This 
is  pseudo-bulbar  paralysis,  which  depends  upon  the  fact  that  small  but  multiple 
lesions  in  both  hemispheres  interrupt  the  course  of  the  nerve  fibers  between 
the  cortex  and  the  bulbar  nuclei  of  the  muscles  of  mastication,  deglutition 
and  speech.  Unilateral  location  of  the  foci  alone  is  so  rare  that  we  need 
not  consider  here  such  cases  described  as  curiosities  only.  This  is  explain- 
able from  the  fact  that  the  lower  facial,  the  hypo-glossal  and  the  motor 
portion  of  the  trigeminus  are  connected  with  both  hemispheres,  ami  that 
as  a  rule  one  of  these  is  sufficient  to  maintain  the  functions  of  the  muscles  sup- 
plied by  these  nerves.  Bilateral  multiple  losses  of  substance  are  seldom 
located  in  the  cortex,  more  frequently  in  the  centrum  ovale,  the  central  ganglia 
(particularly  in  the  putamen  of  the  lenticular  nucleus),  in  the  internal  capsule 
and  in  the  crura  cerebri.  * 

In  typical  cases  of  this  pseudo-bulbar  paralysis- — we  prefer  this  expression 
to  the  more  cumbrous  "cerebral  glosso-pharyngo-labial  paralysis" — the  pa- 
tients have  formerly  suffered  from  the  previously  described  lacunar  hemiplegia. 
Now  comes  a  repetition  of  the  same  attack,  this  time,  however,  on  the  op- 
posite side.  This  shows  us  that  the  arteriosclerosis  lias  led  to  the  gradual 
production  of  small  tissue  defects  in  the  second  hemisphere.  And  now  we 
notice  that  this  second  half-sided  paresis  is  accompanied  by  definite  disturb- 
ances  of  articulation  and  deglutition,  which,  however  (in  contradistinction  to 
the  first  time),  persist  further,  while  the  weakness  of  the  extremities  gradually 
passes  off  this  time  also.  Besides  this  typical  beginning,  as  rarer  methods  of 
occurrence  of  arteriosclerotic  pseudo-bulbar  paralysis  are  observed,  its  sudden 
and  definite  origin  after  a  single  attack;  slow  development  in  steps  broken  by 
remissions;  finally,  a  transition  form  with  abrupt  commencement,  then  total 
disappearance  and  progressiva  reappearance  of  the  symptoms  anew. 

Let  us  look  more  closely  into  this  last.  The  speech  becomes  drawling, 
monotonous,  there  is  sometimes  aphonia.  While  the  formation  of  vowels  suiters 
less,  the  consonants  are  pronounced  badly  and  with  trouble,  so  that  in  severe 
cases  l he  speech  becomes  unintelligible.     In  speaking  the  breath  often  fails,  and 

to  complete  the  sentence  the  patient  must  begin  over  again  several  times;  the 
enunciation  takes  on  a  chopped  off  or  semi-explosive  character.  According  to 
whether  paresis  of  the  lips  or  that  of  the  palate  dominates,  disturbances  in  the 


230  LECTURE    XV 

formation  of  labials  or  nasal  character  predominate.  The  mobility  of  the 
tongue  can  be  so  greatly  affected  that,  following  the  laws  of  gravity,  it  lies 
immovable  upon  the  floor  of  the  mouth.  Usually,  however,  it  can  be  protruded, 
at  least  to  a  certain  extent,  but  there  are  disturbances  in  lateral  movements 
or  in  forming  a  trough,  or  frequent  repetition  of  its  protrusion  leads  to  rapid 
paralysis.  Also  the  movements  of  the  palate  are  either  suspended  or  only 
slow  and  incomplete ;  in  the  larynx,  however,  paresis  of  the  vocal  cords  but 
rarely  reaches  a  high  degree.  The  muscles  of  mastication  are  usually  again 
greatly  affected,  a  close  approximation  of  the  teeth  is  impossible,  the  mouth 
often  remains  permanently  half  open.  Eventual  paralysis  of  the  pterygoids 
manifests  itself  through  the  impossibility  of  pushing  the  lower  jaw  forward 
or  laterally.  Sometimes,  also,  the  muscles  opening  the  mouth  are  weakened 
and  complete  opening,  or  opening  it  against  resistance  is  impossible.  The 
taking  of  nutrition  sometimes  develops  exceedingly  marked  disturbances.  Here 
along  with  weakness  of  the  masticatory  muscles,  that  of  the  muscles  of  the 
tongue,  lips  and  cheeks  plays  a  role;  food  cannot  be  pushed  between  the  teeth, 
falls  out  of  the  mouth,  and  must  often  be  forced  into  the  pharynx  by  the  aid 
of  a  finger.  If  paralysis  of  the  palate  and  of  the  pharynx  are  added,  portions 
of  food  get  into  the  nose  or  the  larynx.  Still,  in  mild  cases,  the  act  of  eating, 
namely,  when  the  patient  eats  slowly  and  selects  solid  and  semi-liquid  food,  is 
relatively  well  accomplished. 

What  constitutes  the  chief  difference  between  the  disturbances  just  related 
and  those  which  we  find  in  true  bulbar  paralysis,  is  the  fact  that  the  paralyzed 
muscles  in  pseudo-bulbar  paralysis  show  no  degenerative  atrophy  and  no  reac- 
tion of  degeneration.  Also,  mutatis  mutandis,  the  same  difference  which  spastic 
spinal  paralysis  presents  in  contradistinction  to  spinal  progressive  muscular 
atrophy.  True  conditions  of  contracture  can,  indeed,  little  by  little  develop  in 
the  lips,  the  tongue  and  the  palate,  in  patients  with  pseudo-bulbar  paralysis. 
Increase  of  the  masseter  reflex  is  scarcely  ever  absent  and  is  understandable. 
Paradoxical,  on  the  other  hand,  is  the  very  frequent  disappearance  of  the 
palate  and  pharyngeal  reflexes  even  when  the  voluntary  movements  of  these 
parts  are  still  intact. 

The  expression  of  countenance  has  something  very  characteristic.  The 
lower  part  of  the  face  acquires  little  by  little  a  mask-like  stiffness  through  the 
combination  of  paralysis  and  contracture.  Saliva  flows  from  the  half  open 
mouth.  The  physiognomy  takes  on  a  lacrimose  expression.  With  this  con- 
trasts, indeed,  the  better  mobility  of  the  forehead,  and  the  movable,  often 
expressive  eyes. 

Much  rarer  than  pseudo-bulbar  paralysis,  is  another  disease  condition  pro- 
duced by  bilateral  disseminated  tissue  defects,  the  senile  incontinence,  type 
Homburger.  One  case  observed  by  me  corresponds  anatomically  exactly  with 
the  statements  of  this  author,  that  is,  there  was  a  symmetrical  status  lacunaris, 
in  the  optic  thalamus  and  in  the  corpus  striatum.  Clinically,  such  cases  are 
characterized  by  the  automatic  character  of  the  discharge  of  urine.  At  more  or 
less  regular  intervals  approximately  the  same  quantity  of  urine,  with  constant 
retention  of  a  residual  portion,  is  discharged,  and  so  suddenly  that  the  patient 
wets  himself.  The  ability  to  urinate  spontaneously  is  preserved  at  the  start 
but  is  lost  later. 


ARTERIOSCLEROSIS    OF   THE    NERVE    CENTERS  231 

After  a  longer  or  shorter  time  all  the  somatic  consequences  of  multiple 
arteriosclerotic  focus  formation  in  the  brain  are  usually  combined  with  a 
psychical  decadence,  becoming  ever  plainer.  We  enter  with  this,  the  subject 
of  the  arteriosclerotic  dementias.  I  intentionally  use  the  plural,  since  quite 
different  symptom-complexes  occur.  In  patients  who  have  suffered  from 
lacunar  hemiplegias,  in  pseudo-bulbar  paralysis  and  in  the  incontinence  just 
related,  we  are  struck  particularly  by  the  triad:  weakness  of  memory,  morbid 
emotivity,  and  loss  of  interest.  The  whole  mental  habitus  becomes  something 
silly.  The  patient,  for  example,  pours  out  hot  tears  for  a  dog  that  was  run 
over,  about  which  he  read  in  the  newspaper,  while  the  fortune  or  misfortune  of 
his  family  does  not  impress  him.  Also  the  hypochondriacal  complaints  which 
are  brought  forward  in  such  numbers  by  these  old  people,  give  the  impression 
of  mental  weakness.  They  do  not,  as  a  rule,  relate  to  serious  troubles,  for 
example,  to  the  loss  of  memory,  power  of  attention,  etc.,  but  to  trivialities,  as 
to  the  hardness  of  their  stools,  etc.  Upon  this  quite  characteristic  basis  but 
in  a  rather  manifold  combination,  a  whole  line  of  further  psychical  anomalies 
develop  themselves,  for  example,  ideas  of  unseen  influence,  suicidal  impulses, 
even  hallucinations,  so  as  observed,  very  heterogenous  individual  pictures  occur. 
Many  of  these  patients  must  be  placed  under  psychiatric  care;  I  will  not  enter 
upon  the  further  disease  pictures  well  known  to  asylum  physicians.  Only  one 
special  arteriosclerotic  psychosis  would  I  mention  here,  since  to  know  and  to 
recognize  its  early  stages  is  of  importance  for  the  non-psychiatrist  also.  I 
mean  "arteriosclerotic  pseudo-paralysis."  (Again  a  "pseudo" — you  see,  arte- 
riosclerosis is  a  great  simulator  among  diseases.)  As  a  fact  the  similarity  of 
this  syndrome  to  mctasyphilitic  dementia  paralytica  is  quite  marked  (see  Lec- 
tin' XIII,  page  205).  One  of  the  newest  descriptions  of  arteriosclerotic  pseudo- 
paresis,  whose  recognition  we  owe  to  Klippel,  Alzheimer  and  Binswanger,  is  by 
Charles  Ladame.  According  to  this  author,  the  clinical  symptoms  of  these 
patients  (they  are  usually  men  between  50  and  (50  years  old)  are  in  the  somatic 
sphere,  premature  senility,  bodily  deterioration,  sluggishness  in  the  pupillary 
reaction  for  light,  convergence  and  accommodation,  exaggeration  of  the  tendon 
reflexes,  hardening  of  the  arteries,  increased  blood  pressure,  sometimes  also, 
arteriosclerosis  of  the  kidneys.  In  the  psychical  sphere  isolated  defects  of 
intelligence,  good  preservation  of  orientation  in  general  and  of  consciousness 
of  self  and  of  illness,  reduction  of  the  power  of  fixing  the  attention,  partial  de 
fects  of  memory;  sometimes  very  good  judgment  forming  capacity  in  certain 
directions,  demeanor  externally  well  regulated  (they  present  a  "deceptive 
front").  Delusional  ideas  (mainly  of  a  hypochondriacal  nature),  illusions  and 
hallucinations,  occur.  The  mood  shows  a  mixture  of  anxiety,  indifference, 
euphoria,  great  egotism,  ideas  of  suicide.  There  is  marked  impulsive  automa- 
tism. The  differential  diagnosis  from  progressive  paralysis  must  usually  be 
based  upon  the  condition  of  the  pupils,  the  defective  menial  condition  with 
retained  personality,  appreciation  of  disease,  the  age  of  the  person  and  his 
genera]  habitus.  The  prognosis  is  in  I  he  great  majority  of  cases  had.  The 
anatomical  substratum  of  this  psychosis  is  not  a  simple  one.  It  can  he  made 
up  of  the  most  varied  results  of  the  ci  rebral  vascular  disease,  including  Marie's 
lacuna',  puncl  tte  or  larger  hemorrhages  of  the  brain,  cortical  or  subcortical 


232  LECTURE    XV 

foci  of  softening,  or  of  the  "subcortical  chronic,  progressive  encephalitis" 
described  by  Binswanger  a  diffuse  degeneration  of  the  medullary  layer  as  a 
consequence  of  insufficient  blood  supply. 

Treatment 

In  conclusion  a  few  therapeutic  indications.  Among  the  different  clinical 
pictures  which  we  have  passed  in  review,  of  course  only  those  which  are  usually 
the  expression  either  of  imperfect  nutrition  or  of  temporary  vascular  spasm, 
present  a  favorable  outlook  as  to  the  possibility  of  being  influenced  by  treat- 
ment. In  the  other  forms,  the  treatment  can  only  seek  the  prophylactic  end  of 
preventing  further  progress  of  the  lesion  if  possible,  but  this  end  is  important 
enough.  Let  me  emphasize  that  in  spite  of  their  organic  basis  many  of  the 
troubles  of  the  arteriosclerotic  pseudo-neurasthenics  are  to  be  favorably  in- 
fluenced by  ps3'cho-therapeutic  reassurance,  since  along  with  the  material  basis 
the  depressing  feeling  of  threatened  "breakdown,"  and  anxiety  about  previously 
unexperienced  disagreeable  sensations  exercise  a  morbid  influence.  Under  all 
conditions,  further,  the  greatest  importance  must  be  laid  upon  the  dietetic 
regime ;  absolute  milk  diet  is  to  be  considered  only  for  a  short  time  and  in  the 
rare  cases  of  particularly  obstinate  sleeplessness  or  headache,  though  it  has 
here  sometimes  a  striking  effect.  In  general  a  bland,  predominantly  ovo-lacto- 
vegetarian  diet  suffices.  We  must  endeavor  to  so  arrange  it  that  the  patient 
will  take  it  for  a  long  time  without  repugnance,  and  hence  must  proceed  not 
too  rigidly.  Meat,  for  example,  may  be  permitted  at  the  mid-day  meal,  but 
only  one  dish,  and  the  wrhite  meats  are  to  be  preferred.  Fresh  water  fish  may  be 
permitted,  sea  fish  forbidden.  Bouillon  should  be  replaced  by  cereal  and 
vegetable  soup ;  plenty  of  vegetables,  easily  digested  farinaceous  foods  and 
potatoes,  fruit  raw  or  cooked,  are  permitted.  Alcoholic  beverages,  strong 
coffee,  and  particularly  the  use  of  tobacco  should  be  restricted  as  much  as  pos- 
sible, and  all  the  stronger  condiments  avoided.  The  patient  should  take  plenty 
of  milk,  eventually  buttermilk,  yoghurt,  kefir.  In  general,  as  table  beverages, 
alkaline  mineral  waters,  as  those  of  Vichy,  Passugg,  Neuenahr,  Fachingen,  etc., 
are  to  be  recommended,  particularly  during  iodide  cures. 

Now,  as  far  as  these  cures  go  you  know  that  of  late  the  indications  for 
iodide  of  potassium  in  arteriosclerosis  have  been  much  narrowed  by  internists. 
We  neurologists,  however,  consider  this  agent  now,  as  formerly,  so  valuable  a 
remedy  that  it  should  not  remain  untried  in  any  form  of  nervous  manifestations 
in  arteriosclerosis.  U/pon  what  the  action  of  iodine  depends  is  not  yet  definitely 
concluded;  it  appears  (from  the  investigations  of  Miiller,  Inada  and  others) 
determined  that  by  reducing  the  viscosity  of  the  blood  it  facilitates  the  flushing 
of  the  nervous  centers;  also  a  certain  vasodilating  action  is  attributable  to  it. 
According  to  Pouchet  it  has  also  a  decidedly  stimulating  effect  upon  the  lympho- 
and  leuko-cj'tes  and  aids  phagocytic  processes,  by  which  a  modern  explanation 
is  furnished  for  its  ancient  reputation  as  a  "summum  resorbens."  Very  ques- 
tionable, on  the  other  hand,  is  the  specific  curative  effect  upon  parts  of  the 
vessel  walls  already  affected  with  arteriosclerosis  affirmed  for  it,  while  again, 


ARTERIOSCLEROSIS    OF   THE    NERVE    CENTERS  233 

a  prophylactic  action  in  this  direction  is  plausible  from  the  fact  that  administra- 
tion of  iodides  often  lowers  the  blood  pressure. 

Since  iodide  of  potassium  or  iodide  of  sodium  must  be  prescribed  always  for 
a  long  time  in  arteriosclerotic  nervous  diseases,  it  is  recommended  that  for  the 
prevention  of  iodism  daily  doses  of  from  0.5  to  1.0  should  not  be  exceeded,  and 
that  after  each  20  days  of  use  there  should  be  a  10  day  pause.  The  addi- 
tion of  sodium  bicarbonate  usually  causes  the  drug  to  be  better  tolerated  by 
patients  with  sensitive  stomachs.  As  a  specially  well  tolerated  combination  I 
would  mention  that  of  potassium  iodide  and  strychnine :  besides  the  alkaline 
iodides,  in  the  better  class  of  practice  a  number  of  organic  preparations  of 
iodine,  such  as  iodipin,  sajodin,  iodon,  etc.,  may  be  used;  also  these  prepara- 
tions are  usually  well  tolerated  and  iodism  is  almost  excluded,  but  in  activity 
they  do  not  compare  with  the  alkaline  iodides. 

While  we  are  speaking  of  drugs  we  may  consider  the  question  of  hypnotics, 
to  use  which  we  are  often  compelled  by  arteriosclerotic  insomnia.  They  appear 
to  me  unobjectionable  provided  that  we  avoid  such  remedies  as  act  unfavorably 
upon  the  vessel  walls  (chloral  hydrate,  amylenhydrate,  paraldehyde,  for  ex- 
ample), and  on  the  other  hand  follow  the  end  of  the  reestablishing  the  ability  to 
sleep  spontaneously.  The  remedies  of  choice  are  the  difficultly  soluble  hypnotics 
of  the  type  of  trional  and  veronal;  we  must  resort  to  them  when  we  cannot 
accomplish  our  ends  with  dietetic  and  hydriatic  measures,  or  with  nightly  doses 
of  bromides  from  1.0  to  2.0  bromide  of  potassium  (we  refrain  from  giving 
larger  doses  of  bromides  since  the  arteriosclerotic  seems  to  have  a  predisposition 
for  bromism).  1.0  (grs.  xv)  of  trional  or  0.5  of  veronal  (gr.  viii)  dissolved  in 
some  hot  liquid  is  given  one  hour  before  bedtime ;  this  medication  can  be  given 
every  evening  if  it  is  desired  that  the  patient  should  get  the  full  hypnotic  effect 
of  the  normal  dose,  and  also  when  necessary,  should  sleep  on  into  the  next  day. 
Under  these  conditions,  after  a  few  days  a  certain  sleepiness  often  appears  and 
this  gives  us  the  signal  for  the  reduction  of  the  dose — of  veronal,  for  example, 
to  0.3  (gr.  5).  Later  it  is  attempted  from  time  to  time  to  intermit  a  night, 
then  to  give  the  hypnotic  every  second,  then  every  third  night,  and  finally  to 
withdraw  it  altogether.  For  senile  "sleep  inversion,"  Hamburger  has  proposed 
a  method  of  "reinversion"  of  the  type  of  sleep  which  I  can  thoroughly  recom- 
iiiiiid :  forcibly  keeping  the  patient  awake  by  day  is  carefully  avoided;  on  the 
other  hand,  he  is  kept  in  bed,  and  we  limit  ourselves  at  first  to  procuring  some 
sleep  at  night  with  hypnotics.  If  now  the  duration  of  the  sleep  by  day  and 
its  depth  diminishes,  the  evening  dose  of  hypnotic  can  be  increased  and  the  pa- 
tient kept  up  longer  and  longer  by  day. 

Of  hydrotherapeutic  measures,  hoi  sail  and  mustard  fool  baths  which  should 
be  used  daily  when  there  is  a  tendency  to  congestions  about  the  head,  vertigo, 
headache,  etc.,  may  be  mentioned  in  the  first  place.  Further  tepid  baths  of 
iml   too  long  duration  and  tepid   rain  douches  uiili   rubbing  down  afterward, 

work  favorably.  Carbonic  acid  baths  should  be  used  only  with  great  caution, 
as  they  are  poorly  borne  by  many  patients.  Rath  (aires  proper,  unless  they  are 
indicated  on  account  of  some  other  result   of  arteriosclerosis  are  not   called   for. 

The  patient  is  best  sent  to  some  quid  resorl  of  medium  altitude  (500  to  <S00 
meters — 1,500  to  2,500  feel )  which  affords  plenty  of  opportunity  for  being  out- 


234  LECTURE    XV 

doors,  is  protected  from  the  wind,  and  has  but  moderate  range  of  temperature. 
Sometimes  general  massage  of  the  body,  after  the  Swedish  method,  acts  quite 
strikingly  both  upon  the  circulatory  conditions  and  upon  the  general  health. 
The  symptomatic  treatment  of  the  spastic  phenomena,  paresis  of  the  muscles 
innervated  from  the  bulb,  incontinence,  etc.,  is  the  same  as  that  already  de- 
scribed in  the  preceding  lectures  for  these  symptoms  from  other  causes. 


LECTURE   XVI 

The  Acute  Infectious  Diseases  of  the  Central 
Nervous  System 

A.     "Essential  Infantile  Paralysis"   (The  Heine-Medin  Disease). 
(Anterior  Polyomyelitis) 

Under  essential  infantile  paralysis  we  include  disease  conditions  which  at- 
tack the  childish  organism  in  stormy  fashion,  to  localize  themselves  shortly  in 
definite  parts  of  the  central  nervous  system  (in  the  vast  majority  of  cases  in 
tin  anterior  horns  of  the  spinal  cord)  and  there  to  lead  to  characteristic  in- 
flammatory  foci  with  subsequent  degenerative  processes.  The  clinical  corollary 
of  these  lesions  consists,  as  a  rule,  in  flaccid  paralyses  which  show  no  tendency 
to  progression,  but,  on  the  contrary,  usually  undergo  decided  regression,  soon 
after  the  onset  of  the  disease.  The  disease  often  occurs  in  epidemic  form;  it  has 
Been  produced  experimentally  in  animals.  While  the  first  accurate  description 
<:f  the  disease  originated  with  the  Stuttgart  physician  Jakob  r.  Heine  (1840), 
tlir  Stockholm  pediatrist  0.  Media,  on  the  basis  of  careful  studies  on  the  Swedish 
epidemics  of  LSS7  and  1895,  first  showed  with  all  definiteness  the  infectious 
nature  of  the  disease. 

The  classical,  until  a  short  time  ago,  almost  exclusively  considered,  form 
of  the  Heine-Medin  disease  is  infantile  spinal  paralysis  (poliomyelitis  anterior 
acuta  infantum).  This  includes  over  90  per  cent,  of  all  the  sporadic  and  epi- 
demic cases  of  this  nosological  group.  We  are  hence  certainly  justified  in  our 
clinical  discussion,  in  keeping  in  mind  at  first  this  typical  manner  of  occurrence. 
Only  afterward  will  the  varieties  of  the  disease  due  to  variation  in  the  location 
of    the    anatomical    substratum,    hi'    considered. 

Course  (Hid  Symptomatology 

While  in  general  the  separation  from  each  other  of  different  stages  of 
disease  in  affections  of  the  nervous  system  is  rather  artificial,  and  the  boundaries 
between  these  stages  can  scarcely  he  drj  wn  sharply,  in  acute  anterior  poliomye- 
litis we  can  without  doing  violence  to  the  facts  hold  fast  to  the  usual  dill'eieii; 
tiation  into  four  periods,  the  infectious  stage,  tin-  paralytic  stage,  the  stage  of 
regression,  and.  finally,  the  stage  of  trophic  disturbances  and  contractures. 

1.     INFECTIOUS    STAGE 

The  age   at    which    poliomyelitis   acuta    anterior  occurs   by    preference    is    the 
Bcond  year  of  life.     It  finds,  however,  relatively  many  victims  also  in  the  third 


236  LECTURE    XVI 

and  fourth  years.  The  morbidity  then  rapidly  decreases  so  that  cases  be- 
ginning between  the  8th  year  and  puberty  are  comparative  rarities.  The  first 
year  of  life  also  furnishes  but  a  very  small  contingent. 

The  first  signs  of  the  disease  have  so  little  that  is  characteristic  that  in  the 
first  stage  the  diagnosis  can  only  be  made  definitely  when  the  case  occurs  while 
an  epidemic  of  the  Heinc-Mcdin  disease  prevails.  The  children  fall  ill  with  rap- 
idly rising  fever,  which,  however,  only  exceptionally  exceeds  40°  C.  (104°  F.), 
become  somnolent  (but  scarcely  ever  comatose),  often  show  vomiting  and 
diarrhoea,  not  rarely  coryza  or  angina.  Sometimes  the  children  complain  of 
headache  and  pain  in  the  back  or  limbs,  frequently  there  is  so  much  hyperesthesia 
of  the  integument  that  they  scream  when  taken  hold  of.  A  sensitiveness  to 
pressure  and  to  tapping  over  the  spinous  processes,  with  stiffness  of  the  spine 
is  frequent  (perhaps  to  be  considered  as  a  local  symptom).  Further,  frequent 
disease  manifestations  of  the  infectious  stage  are  convulsions,  twitching,  grind- 
ing the  teeth,  rolling  the  eyes,  profuse  sweating. 

Ed.  Midler  attributes  great  importance  to  the  absence  of  leucocytosis  in  the 
blood  picture,  in  the  diagnosis  of  the  febrile  stage  of  acute  poliomyelitis,  in- 
deed, he  has  found  usually  decided  leucopenia,  a  reduction  of  the  number  of 
leucocytes  to  from  3,000  to  5,000.  For  him,  leucopenia,  hyperidrosis  and 
hyperesthesia  represent  the  three  cardinal  symptoms  of  the  early  stage  of  es- 
sential infantile  paralysis,  a  view  whose  justification  is,  however,  not  yet  fur- 
nished. On  examination  of  the  spinal  fluid,  however,  nothing  definitely  char- 
acteristic has  been  found;  sometimes  there  is  moderate  lymphocytosis,  sometimes, 
on  the  conti'ary,  leucocytosis  with  almost  complete  absence  of  lymphocytes.  The 
duration  of  the  infectious  or  febrile  stage  is,  as  a  rule,  from  24  to  48  hours. 


2.     THE   STAGE   OF   PARALYSIS 


In  typical  cases,  the  stage  of  paralysis  in  acute  poliomyelitis,  abruptly 
terminates  its  febrile  early  period.  The  temperature  falls,  paralytic  symptoms 
immediately  appear,  and  within  a  few  hours  reach  their  maximum  in  intensity 
as  well  as  in  extent.  This  last,  however,  varies  within  wide  limits.  Most  fre- 
quently the  paralysis  assumes  a  paraplegic  type,  that  is,  affects  both  lower  ex- 
tremities; there  follow  according  to  frequency,  paralysis  of  a  single  leg  (mono- 
plegia cruralis),  that  of  one  arm  (monoplegia  brachialis),  that  of  both  arms 
(diplegia  superior);  further,  tetraplegia,  triplegia,  and  finally,  hemiplegia, 
which  latter  is  denominated  "alternating"  when  it  affects  the  right  arm  and  left 
leg,  or  vice  versa.  The  trunk  and  neck  muscles  are,  as  a  rule,  unaffected;  still, 
upon  careful  examination  of  the  abdominal  muscles  a  unilateral  or  bilateral 
paralysis  of  them  is  not  rarely  observed,  in  the  first  case  the  umbilicus  is  drawn 
toward  the  healthy  side. 

Cases  in  which  the  cranial  nerves  are  also  paralyzed  (eye  muscles,  facial, 
hypoglossal)  naturally  extend  beyond  the  bounds  of  anterior  poliomyelitis  into 
those  of  polioencephalitis  ;  a  more  extensive  affection  of  the  motor  nuclei  of  the 
brain  axis  can,  indeed,  lead  to  rapid  exitus  from  respiratory  or  cardiac  paralysis. 

This  paralysis  is  now  a  thoroughly  flaccid  one,  and  can  through  the  high 
degree   of  this   hypotonia,   change   the   extremities    into   unresisting,    dangling 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     237 

appendages,  into  "jumping  jack  limbs"  ("membres  de  polichinelle").  The 
reflexes  in  the  affected  muscles  are  of  course  lost.  Typical,  further,  is  the  escape 
of  the  bladder  and  the  rectum  ;  where  any  incontinence  or  retention  of  urine  and 
feces  is  noted,  it  is,  as  a  rule,  an  inconsiderable  and  temporary  phenomenon. 
Objective  testing  of  sensibility  discloses  no  anomaly;  on  the  other  hand,  older 
children  sometimes  complain  of  severe  pains  in  the  affected  limbs.  These  pains, 
which  we  consider  as  due  to  irritation  by  the  inflammatory  focus  of  the  pain 
fibers  which  cross  in  the  gray  commissure  (see  Lecture  X,  page  145),  have 
as  an  evidence  of  the  intensity  of  this  inflammatory  process,  a  prognostically 
unfavorable  indication.  The  paralytic  symptoms  usually  remain  for  some  days 
in  unaltered  intensity  and  extent;  then,  however,  the  spontaneous  occurrence  of 
marked  improvement  announces  the  beginning  of  the  third  typical  phase  of  the 
disease. 

3.     THE    STAGE    OF   REGRESSION 

It  is  noticed  that  in  single  muscles  of  the  paralyzed  extremities  the  power 
of  voluntary  movement  appears  again,  so  that  the  extent  of  the  paralysis,  as 
compared  to  the  picture  presented  in  the  preceding  stage,  slowly  diminishes, 
or,  as  it  is  usually  expressed,  the  paralysis  "concentrates  itself."  Even  before 
this  regression  or  concentration  has  reached  its  maximum,  however,  the  study 
of  the  electrical  reactions  permits  us  to  predict  which  muscle  groups  will  most 
rapidly  regain  their  function,  and  in  which  others  the  paralysis  will  continue 
Bngest,  or  will  declare  itself  permanent.  The  seriously  or  incurably  affected 
muscles  namely,  show  early  (from  the  10th  to  the  14th  day  after  the  beginning 
of  the  disease)  reaction  of  degeneration,  and  in  general,  one  can  hold  fast  to 
the  rule  given  by  Duchenne,  that  muscles  which  3  weeks  after  the  onset  of  the 
acute  spinal  paralysis  no  longer  react  to  faradism  must  be  considered  as  lost. 
Exceptions  to  this  rule  I  have  indeed  occasionally  had  the  opportunity  of  ob- 
serving, and  otherwise  also,  there  are  sometimes  findings  which  do  not  corre- 
spond to  the  general  principles  of  electro-diagnosis,  in  that,  for  example,  mus- 
cle-, permanently  paralyzed  show  only  a  qualitative  reduction  of  galvanic  and 
faradic  irritability  which  continually  progresses  to  entire  loss  of  reaction.  The 
regression  which  probably  occurs  from  disappearance  of  the  inflammatory 
oedema  and  of  the  infiltrative  processes,  can  proceed  to  complete  recovery;  still, 
such  cases,  which  are  somewhat  more  frequently  observed  in  epidemics  of  the 
Meine-Medin  disease  than  among  sporadic  cases  of  essential  infantile  paralysis, 
form  only  a  small  minority.  In  general,  namely,  acute  anterior  poliomyelitis 
after  greater  or  less  "concentration"  leaves  behind  as  corollary  of  the  total 
destruction  of  definite  centers  of.  the  anterior  horns,  the  final  elimination  of 
certain  muscles.  There  is,  indeed,  a  certain  regularity  in  the  choice  of  locali- 
sation of  the  residuary  muscular  paralysis.  In  the  upper  extremities,  most 
frequently  permanently  affected  are  the  deltoid,  the  infraspinatus,  the  serratus, 
the  trapezius,  the  teres  minor.  In  the  lower  extremities,  the  quadriceps,  par- 
ticularly,  however,    the    extensors    of    the    foot    and    toes.       We   will    consider    the 

deformities  to  which  these  paralyses  lead,  on  describing  the  next  stage.  Corre- 
sponding to  its  anatomical  substratum   (the  destruction  of  the  tropho-motor 


238 


LECTURE    XVI 


centers  of  the  anterior  horn)  the  disease  leads  to  rapid  progressive  degenerative 
atrophy  of  the  affected  muscles,  whose  contractile  substance  finally  is  entirely 
replaced  by  fat  and  connective  tissue.  Upon  superficial  observation  of  the 
little  patient  during  the  stage  of  regression  a  decided  subcutaneous  development 
of  fat  in  the  paralyzed  region,  predominating  to  a  certain  extent  over  the 
muscular  wasting,  may  lead  to  deception.      Upon  an  average  the  "concentra- 


Fig.  70. 
Pes  Equinovarus  as  a  Result  of  Anterior  Poliomyelitis. 

tion"  of  the  paralysis  is  completed  after  8  or  10  months,  though  a  much  longer 
extent  of  the  stage  of  regression  (up  to  a  year  and  a  half,  or  more)  is  not  at 
all  rare. 


4.     STAGE   OF   TROPHIC   DISTURBANCES   AND    CONTRACTURES 

The  stage  of  regression  passes  over  now  into  the  stationary  and  final  period 
of  poliomyelitis,  in  which  the  bodily  deformities  resulting  from  the  combination 
of  paralysis,  contracture  and  atrophy  dominate  the  clinical  picture.  The  de- 
generative muscular  atrophy  reaches  its  highest  degree  which  no  subcutaneous 
development  of  fat   can  mask   any  longer,   so  that  the  limbs   affected   appear 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     239 


markedly  thinned,  in  part,  of  skeleton-like  emaciation.  At  the  same  time,  severe 
disturbances  of  nutrition  affect  the  bones  of  these  extremities;  these,  in  the 
further  growth  of  the  child,  remain  far  behind  those  of  the  healthy  limbs  in 
development,  and  as  the  X-ray  study  of  such  cases  shows,  there  may  be  re- 
gressive and  degenerative  processes  of  their  cortical  layers,  which  in  time  may 
become  so  thin  that  very  slight  causes  lead  to  the  so-called  spontaneous  frac- 
tures. Also  in  the  ligaments  there  are  atrophic  processes  which  may  lead  to  an 
excessive  relaxation  of  the  joint  (flail  joint),  so,  for  example,  in  the  shoulder- 
joint,  the  head  of  the  humerus  is  seen  to  fall  out  of  its  socket,  the  natural  round- 
ing of  the  shoulder  is  interrupted  by  a  deep  furrow,  and  the  palpating  finger 
can  easily  be  pressed  in  between  the 
scapula  and  the  joint  surface  of  the 
humerus.  The  separation  of  the  joint 
surfaces  from  one  another  leads  in  time 
to  deformities  which,  however,  never  reach 
such  a  degree  as  in  syringomyelia  and 
tabes  dorsalis.  Of  great  practical  im- 
portance are,  further,  the  shortening  con- 
tractures which  occur  in  the  unparalyzed 
(or  only  partially  paralyzed)  antagonists 
of  the  totally  paralyzed  muscles,  as  ex- 
pression of  the  mechanical  and  trophic 
predominance  which  the  unaffected  or 
relatively  unaffected  muscles  attain  in 
time.  Through  this  there  arise  character- 
istic anomalies  of  position,  so,  for  ex- 
ample, pes  equinovarus  which,  by  far  the 
most  frequent  deformity,  occurs  on  ac- 
count of  contracture  of  the  tibialis  and 
the  triceps  sura;,  flic  peronei,  and  extensor 
of  the  toes  ami  great  toe  being  eliminated. 
In  degeneration  of  the  calf  muscles,  on 
tin-  other  hand,  there  is  pes  calcaneus. 
preponderance  of  the  muscles  of  the  solo 
over  the  more  or  less  weakened  anterior 
and   posterior  muscles  of  the  leg,  leads  to 

"paralytic  pes  cavus,"  atrophy  of  the  peroneal  muscles,  Hie  anterior  tibial 
being  preserved,  to  pes  valgus.  In  the  hand,  infantile  paralysis  but  rarely 
lead-,  to  characteristic  anomalies  of  position,  if  at  all  to  "claw  hand"  de 
Scribed  in  Lecture  VI,  page  108.  .Rather  frequent  is  scoliosis,  which  is  to  he 
considered  sometimes  as  a  primary,  sometimes  as  a  secondary  result  of  polio- 
myelitis. The  first  is  usual;  the  lateral  curvature  of  the  spinal  column  arises 
from  tin'  necessity  of  compensating  the  shortening  of  one  leg,  by  altering  the 
position  of  the  center  of  gravity;  tin'  latter  can  result  from  the  comparatively 
rare  atrophic  paralysis  of  the  muscles  "(  the  hack.  With  the  scoliosis  there 
are  often  deformities  of  the  pelvis,  which  in  female  patients  may  in  later  years 
lead  to  serious  difficulties  in  parturition. 


Paralytic  Pes  Cavus  as  ;i   Result  of 
Anterior  Poliomj  elitis. 


24-0 


LECTURE    XVI 


In  the  skin  of  the  paralyzed  extremities  also,  there  are  atrophic  and  vaso- 
motor anomalies.  It  is  cool,  usually  livid  in  color  or  marbled,  thin  and  smooth ; 
often  its  hair  is  abnormal.  The  repeatedly  made  observation  that  the  exanthem 
of  measles  and  scarlet  fever  in  children  who  have  had  poliomyelitis  avoids  the 
paralyzed  limbs,  is  interesting.     The  integument  of  such   individuals  shows   a 


Fig.  72. 

Infantile  Spinal  Paralysis.    Late  Stage;  Extreme  Atrophy  of  the  Lower  Portion  of  the  Trunk, 
the  Pelvic  Region  and  the  Legs;  Marked  Pes  Varus,  Right  Side. 


great  tendency  to  form  bed  sores,  which  furnishes  a  warning  as  to  very  careful 
application  of  orthopedic  apparatus,  if  it  is  needed.  There  is  also  a  marked 
tendencv  to  chilblains. 


ANOMALIES  IN  THE  COURSE  OF  ACUTE  ANTERIOR  POLIOMY- 
ELITIS AND  OTHER  VARIETIES  OF  THE  HEINE-MEDIN 
DISEASE 

Along  with  the  just  described  "classical"  picture  of  acute  atrophic  spinal 
paralysis  in  childhood,  there  are  observed  not  infrequently  anomalies  in  its  course 
which  are  characterized  by  the  absence  of  some  of  the  disease  stages.  We  have 
already  mentioned  the  mild  cases  in  which  the  stage  of  regression  goes  on  to 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     2il 

complete  recovery,  so  that  the  period  of  trophic  disturbances  and  contractures 
does  not  occur;  these  are  the  so-called  "temporary  infantile  spinal  paralyses." 
It  can  further  happen  that  a  "concentration"  of  the  paralysis  does  not  occur, 
in  that  the  paralysis  at  once  establishes  itself  within  its  final  limits  (in  these 
cases  usually  relatively  moderate).  Most  frequently,  however,  the  infectious 
stage  entirely  escapes  clinical  recognition.     Here  belongs  the  so-called  "morning 


Fig.  73. 

Infantile  Spinal  Paralysis.    So-called  "Hand  Walker"  as  a  Result  of  Severe  Dorsal  and 
Lumbar  Poliomyelitis. 

paralysis"  in  which  the  child  is  put  to  bed  healthy  and  lively,  and  upon  awaken- 
Jlig  is  found  paralyzed. 

Less  frequenl  than  the  just  mentioned  anomalies  in  course,  are  those  cases 
in  which  the  same  infectious  agents  which  lead  to  acute  anterior  poliomyelitis 
on  account  of  another  anatomical  localization  of  the  inflammatory  process,  pro- 
duce different  clinical  pictures.  ,()f  course,  their  identity  with  the  typical  in- 
fantile spinal  paralysis,  as  long  as  we  are  unable  Id  recognize  bactcriologically 
their  cause,*  cannot  he  proved  in  the  sporadic  cases,  it  is  shown  much  more, 
from    the   observation   of  such   cases    in   epidemics   of   the   II liiw-Miilhi   disease. 


'Serological  diagnosis  after  Romer,  \<it<r,  Levaditi,  and  others,  does  nut  come  into  prac- 
tical consideration.  Vccording  to  these  authors,  the  blood  of  individuals  affected  with  polio- 
myelitis, or  disease  etiological!)1  related  i"  It,  contains  specific  anti-bodies  which  are  able  to 
neutralize  an  emulsion  prepared  from  nerve  centers  containing  tin-  virus  el'  this  disea  e,  in 
bperiments  upon  nkeys. 


242  LECTURE    XVI 

We  must  thank,  above  everybody  else,  the  Swedish  observer,  Wickman,  for 
our  knowledge  of  these  rare  localizations  of  the  poliomyelitis  virus;  still,  before 
him,  S.  Auerbach,  P.  Marie,  Striimpell  and. others  have  affirmed  the  etiological 
identity  of  certain  meningeal  or  cerebral  diseases  with  epidemic  spinal  paralysis. 

In  the  meningitic  form  the  original  clinical  picture  of  meningitis  can  either 
be  complicated  later  with  poliomyelitic  paralyses  or  there  can  be  the  simple  and 
persistent  picture  of  a  meningitis  which  in  autopsies  by  Wickman  proved  to  be 
serous  meningitis.  This  author  has  successfully  opposed  the  view  championed 
by  several  observers,  for  example,  Pierre  Marie,  according  to  which  there  is  an 
etiological  connection  between  essential  infantile  paralysis  and  epidemic  cerebro- 
spinal meningitis  which  we  will  study  shortly   (see  page  248). 

In  the  cerebral  form,  instead  of  poliomyelitis,  there  is  '"polioencephalitis," 
which  produces,  in  an  acute  manner,  the  picture  of  "infantile  cerebral  paralysis" 
which  will  be  described  at  length  in  Lecture  XXII.  It  need  only  be  emphasized 
here  that  the  cerebral  form  of  the  Heine-Medin  disease  usually  begins  acutely 
with  vomiting,  convulsions,  stupor  and  fever,  from  which,  after  a  few  days, 
the  children  recover,  but  with  a  spastic  hemiplegia  which,  as  a  rule,  is  more 
marked  in  the  arm  than  in  the  leg.  The  tendon  reflexes  are  exaggerated, 
Babinski's  reflex  is  present,  and  in  time,  contractures  appear,  etc.  Not  rarely 
there  occur  later  in  such  children  the  phenomena  of  hemiathetosis  and  hemi- 
ehorea  already  described  in  Lecture  V,  as  well  as  epileptic  attacks.  The 
paralysis  itself  is  never  complete;  rather  is  it  a  more  or  less  marked  paresis. 
Also  it  is  never  accompanied  by  reaction  of  degeneration  and  degenerative 
atrophy,  but  only  with  non-degenerative  "cerebral"  atrophy.  The  bones  of 
the  affected  extremities  are  somewhat  retarded  in  development  and  the  skin 
shows  similar  but  much  less  intense  trophovasomotor  disturbances  than  those 
in  acute  poliomyelitis  anterior. 

Another  variety  takes  the  form  of  ascending  or  Landry's  paralysis,  in  which, 
in  rapid  succession,  the  lower  extremities,  the  trunk  and  the  arms  are  attacked, 
and  finally,  the  respiratory  centers  are  paralyzed.  Almost  all  of  those  cases  of 
infantile  paralysis  which  end  fatally  ("quite  few")  run  their  course  under  this 
picture.  In  this  connection  must  be  mentioned  further,  the  already  shortly 
sketched,  bulbo-pontine  form  in  which  the  facial,  the  hypoglossal  and  the  eye 
muscle  nerves  are  also  affected;  this  form  can  also  occasionally  manifest  itself 
as  a  true  acute  bulbar  paralysis.  Further,  there  is  a  cerebellar  form  of  the 
Heine-Medin  disease  to  which  particularly  Oppcnheim  has  directed  attention, 
and  which  appears  as  an  acute  cerebellar  ataxia,  and  finally,  a  polyneuritic 
form,  in  which  sensitiveness  to  pressure,  and  swelling  of  the  nerve  trunks,  severe 
spontaneous  pains,  paresthesias,  sensory  disturbances,  etc.,  can  be  recognized. 

Etiology  and  Pathological  Anatomy 

The  bacteriology  of  acute  infantile  paralysis  has  so  far  produced  no  useful 
results,  since  the  finding  of  cocci  and  diplococci,  reported  by  former  authors 
were  not  obtained  under  such  precautions  that  the  causal  connection  between 
these  microbes  and  the  spinal  cord  affection  must  be  accepted.  Also  the  newest 
investigations  of  Fleamer  and  Leuis  and  of  Lcraditi  only  permit  one  thing  to 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     243 

be  definitely  affirmed ;  namely,  that  the  cause  of  the  Heine-Medin  disease  must 
be  an  excessively  minute  organism  which  can  pass  through  a  Berkefcld  filter.* 

On  the  other  hand,  the  communicability  of  the  disease  has  been  proved  by 
the  investigations  of  Landsteiner,  Flcxner,  Levaditi,  Romer,  and  others.  If  an 
emulsion  prepared  from  the  spinal  cord  of  a  child  dead  of  acute  poliomyelitis, 
is  injected  into  the  peritoneum,  a  vein,  or  better,  into  the  brain  of  a  monkey, 
the  animal  becomes  paralyzed  and  his  spinal  cord,  prepared  in  an  emulsion, 
develops  the  same  pathogenic  peculiarities  toward  others  of  his  kind.  Other 
animals,  however,  are  almost  without  exception,  immune ;  only  in  the  rabbit 
can  the  disease  be  here  and  there  produced.  The  experimental  poliomyelitis 
or  spinal  paralysis  of  monkeys  presents  its  first  symptoms  from  7  to  10  days 
after  the  injection.  The  stage  of  infection  usually  shows  itself  as  a  general 
tremor,  to  which  flaccid  paralysis  of  the  legs  which  may  extend  to  the  arms, 
trunk  and  the  neck,  is  soon  added.  The  question  as  to  the  natural  port  of  entry 
of  the  Heine-Medin  infection  in  man  is  not  yet  entirely  explained.  Probably 
the  exciting  agent  enters  through  the  mucous  membrane  of  the  pharynx,  the 
nose  or  the  intestinal  canal  and  makes  its  way  along  the  nerve  trunks  to  the 
central  organs. 

Of  the' anatomical  changes  which  it  there  causes,  we  will  keep  in  mind  only 
anterior  poliomyelitis  as  the  typical  lesion;  this  shows  itself  as  a  hematogenous 
myelitis  which  localizes  itself  exclusively,  or  almost  exclusively,  in  the  territory 
(if  the  anterior  spinal  artery.  This  artery  sends  a  special  branch  into  the 
anterior  horn  alternately  according  to  level,  into  the  right  or  into  the  left. 
This  bears  the  name  of  Arteria  sulco-commissuralis  and  forms  within  the 
anterior  horn  an  exceedingly  rich  and  luxuriantly  branched  network,  which 
surrounds  the  group  of  root  cells  in  basket  fashion.  The  greatest  part  of  the 
posterior  horn,  the  white  matter,  as  well  as  the  most  peripheral  portion  of 
the  anterior  horn  near  the  boundaries  of  the  white  columns,  are,  on  the  other 
hand  (by  means  of  radiating  brandies)  nourished  from  another  vascular  sys- 
tem, the  "vasa  corona";  as  the  arterial  crown  of  vessels  which  uniting  the  region 
of  the  anterior  and  that  of  the  posterior  spina]  artery  surrounding  the  periph- 
ery of  the  spinal  cord  are  called  (see  Fig.  74).  Investigations  by  I.tiiitif, 
Rothmann,  and  others,  have  shown  that  a  suspension  of  lycopodium  injected 
into  tin-  abdominal  aorta  in  animals  almost  exclusively  passes  into  the  sulco- 
BOmmissural  artery  and  its  branches,  which  in  a  certain  sense  acts  as  "an  em- 
bolus catcher."  These  experiments  make  it  comprehensible  that  it  can  also 
be  a  "bacteria  catcher"  and  the  particular  vulnerability  of  the  anterior  horn 
to  tin-  cause  of  poliomyelitis  may  he  connected  with  these  peculiarities. 

In   the   first    stages   of  acute  anterior   poliomyelitis,   there   are   found,   as 
Wickman  and  I.  among  others, -have  shown,  a  gnat   infiltration  of  small,  round 
cells  about   the  vessels  of  tin    anterior  horn,  and  smaller  or  larger  extravasa 
tions  of  blood.  The  gray  matter  appears  pale,  its  medullated  fibers  swell  up  and 


rii  Jin  r  .ukI  .v  oguchi,  in  1913,  described  ;i  very  small  globular  organism  which  thej  had 
succeeded  in  cultivating  from  tin-  central  nervous  system  in  monkeys  and  in  human  beings 
.'ililietcil  with  poliomyelitis,  and  in  a  verj  receni  communication  announce  thai  by  a  special 
procedure  they  have  preserved  cultures  of  this  organism  for  eight  months  and  hive  found  it 
^lill  capable  of  producing  poliomyelitis  in  monkeys,  (Journal  of  Experimental  Medicine, 
October,    1913,    and    January,    1915.)— Translator. 


2ii 


LECTURE    XVI 


become  varicose,  or,  on  the  contrary,  they  disappear.  Sometimes  also  necrotic 
foci  filled  with  granular  cells,  round  cells,  and  all  sorts  of  detritus,  are  found. 
A  greater  or  less  number  of  the  motor  cells  of  the  anterior  horn  show  decided 
alterations:  now  they  are  formless,  decidedly  swollen  and  no  processes  are 
recognizable  any  longer ;  again,  they  do  not  take  any  stain,  appear  nearly 
homogeneous  "shadowy";  still  again,  there  is  definite  destruction  of  the  Nissl 
granula  or  "tigroid  scales"  (the  so-called  "tigrolysis")  ;  finally,  sometimes, 
there  is  marked  atrophy,  so  that  the  cells  are  reduced  to  small,  easily  over- 
looked clumps  ("pyknosis"). 

Naturally,  cases  of  infantile  spinal  paralysis  in  which  the  disease  has  ran 
its  course,  come  much  more  frequently  to  autopsy  than  fresh  ones.     Here  there 


n"p 

w 

't 

1 ,/  3 

1 

*     ' 

\ 

V 

>■ 

f 

\ 

£? 

The  Blood  Supply  of  the  Spinal  Cord.     Injected   preparation.      (Carmine  gelatine.) 

is  replacement  of  the  parts  formerly  affected  by  the  inflammation  by  a  more  or 
less  thick,  gliotic  scar  tissue,  more  or  less  rich  in  vessels.  The  more  extended 
the  affection  of  an  anterior  horn  has  been,  the  more  shrunken  it  appears,  on 
which  account  sometimes,  one-half  of  the  spinal  cord  appears  smaller  than 
the  other.  Only  a  more  or  less  considerable  part  of  the  cells  of  the  anterior 
horn  remain  of  characteristic  appearance ;  the  others  have  either  entirely  dis- 
appeared or  have  been  changed  to  inconsiderable  clumps   (see  Fig.  75). 


Prognosis 

The  prognosis  as  to  life  is  in  general,  and  if  the  sporadic  cases  are  chiefly 
considered,  favorable.  Only  rarely  does  exitus  occur  in  the  infectious  stage 
under  hyperpyrexia  and  heart  weakness,  also  the  bulbar  or  ascending  forms 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     245 

of  poliomyelitis  as  well  as  the  meningitic  varieties  of  the  disease,  all  of  which 
endanger  life  through  their  localization,  belong  to  the  exceptions.  In  the 
epidemic  poliomyelitis,  however,  the  prognosis  is  much  more  serious.  So,  ac- 
cording to  Harbitz,  Scheel  and  Wickman,  in  the  epidemics  in  Sweden  and 
Norway  in  1905,  among  2,078  cases  there  were  290  deaths,  which  is  a  mortality 
of  about  1-1  per  cent.  As  to  the  prognosis  with  regard  to  recovery,  complete 
restoration  is  a  great  rarity ;  much  more  frequently  there  is  partial  recovery, 
that  is,  extensive  improvement.    The  more  rapid  the  regression  of  the  paralysis, 


Fig.  75. 

Acute  Anterior  Poliomyelitis  in  Childhood. 

Late  stage,  carmine  preparation  from  the  spinal  cord  of  a  seventy-year-old  man.     (The  cells  of 
the  anterior  horn,  still   recognizable  microscopically,  are   brought  out   by   retouching.) 

the  better  the  outlook  for  a  favorable  result.  Of  the  greatest  importance, 
further,  as  already  emphasized  (page  2.'J7),  is  the  result  of  the  electrodiag- 
nostic  testing.  Permanent  severe  paralysis  are  absent  in  only  about  a  third 
of  the  cases. 

In  various  instances  the  occurrence  of  another  spinal  cord  disease  in  later 
life  in  patients  who  have  had  infantile  spinal  paralysis  has  been  observed  (com- 
bined system  diseases,  diffuse  myelitis;  particularly,  however,  spinal  progres- 
sive  muscular  atrophy   and   chronic   anterior   poliomyelitis).' 


*  Chronic  or  subacute  anterior  poliomyelitis,  an  exceedingly  rare  disease,  which  we  have 
already  mentioned  under  the  differential  diagnosis  of  spinal  progressive  muscular  atrophy,  can 

occur  also  without   previous  acute  poliomyelitis,  and    usually   in  middle  life.      Its  etiology   is  ob- 
scure.     Without    febrile    s\  mptoins,    pain,    sensory    or    sphincter    disturbances,    but    usually    with 

fibrillary  contractions,  there  appears  an  Increasing  weakness  in  the  lower  extremities,  which 


246  LECTURE    XVI 

Differential  Diagnosis 

Most  important  from  a  prognostic  point  of  view  is  the  distinction  between 
poliomyelitis  and  polyneuritis,  since  the  last  (which  is  not  so  rare  in  children 
as  was  formerly  assumed)  presents  a  good  outlook  as  to  complete  recovery. 
In  poliomyelitis,  the  paralysis  reaches  its  maximum  in  a  few  hours,  shows  from 
then  on  a  decided  tendency  to  regression  and  is  scarcely  ever  progressive.  On 
the  other  hand,  the  paralysis  in  acute  polyneuritis  only  reaches  its  maximum, 
as  a  rule,  after  days  or  weeks.  Continuous  extension  or  advance  by  stages 
in  acute  atrophic  spinal  paralysis  is  of  the  greatest  rarity,  on  the  other  hand. 
One  can  count  less  upon  the  results  of  testing  sensibility,  since  it  should  be 
remembered  that  the  polyneuritis  of  childhood  is  not  rarely  purely  motor  (see 
page  -tl).  Decisive  as  to  polyneuritis  is  palpable  thickening  of  nerve  trunks 
or  the  occurrence  of  ''glossy  skin."  The  involvement  of  the  cranial  nerves 
speaks  on  the  whole  more  for  polyneuritis,  optic  neuritis  almost  certainly  so. 
Affection  of  the  muscles  of  the  neck  and  trunk,  in  case  there  is  no  history  of 
diphtheria,  speaks  for  poliomyelitis  and  against  polyneuritis.  In  the  first  the 
decided  affection  of  the  roots  of  the  extremities  is  more  frequent  than  in  the 
last,  which  usually  more  markedly  affects  their  ends.  While  after  about  one 
year  poliomvelitic  paralysis  has  almost  always  reached  its  stationary  stage,  the 
regression  of  polyneuritic  paralysis  can  extend  over  a  much  longer  time.  In 
poliomvelitic  paralysis,  atrophy,  and  reaction  of  degeneration  are  usually  de- 
veloped proportionately.  In  polyneuritis  reaction  of  degeneration  can  be 
found  even  in  muscles  which  can  still  be  moved  voluntarily,  hence  electroprog- 
nosis  is  little  certain  here.  The  reappearance  of  faradic  irritability  which  has 
been  lost,  turns  the  balance  in  favor  of  the  neural  seat  of  the  paralysis.  Oedema 
speaks  for  polyneuritis,  and,  finally,  in  this  last,  the  fever  may  keep  up  longer 
and  reappear  from  time  to  time. 

Acute  myelitis  occurs  exceedingly  seldom  in  children;  in  differential  diag- 
nosis it  hence  plays  a  role  almost  only  as  opposed  to  the  acute  poliomyelitis 
of  adults  ("acute  atrophic  spinal  paralysis"  of  adults).  This  last  affection 
is  exceedingly  inferior  in  frequency  to  infantile  poliomyelitis,  but  nevertheless 
is  not  such  a  very  great  rarity  and  has  occasionally  been  confirmed  by  autopsy 
{Striimpell,  Schultse,  Williamson  and  others).  That  occasionally  during  epi- 
demics of  the  Heine-Medin  disease  cases  affecting  adults  have  been  observed 
justifies  us  in  considering  the  poliomyelitis  of  adults  in  connection  with  in- 
fantile spinal  paralysis.  Many  times,  however,  the  acute  atrophic  spinal  par- 
alysis of  adults  has  been  observed  in  connection  with  influenza,  gonorrhoea, 
puerperium,  typhoid,  etc.,  so  that  perhaps  the  most  varied  agents  come  into 
question   in   connection   with   it.      Clinically   it   differs    from   the   infantile    form 

in  the  course  of  several  days  or  weeks  increases  to  an  atrophic  paralysis.  The  arms,  usually 
soon  after,  share  the  same  fate;  the  condition  remains  stationary  several  months,  then  im- 
proves gradually;  indeed,  there  may  he  complete  recovery.  Important  in  prognosis  is  the  degree 
of  reaction  of  degeneration  (always  to  he  found).  Death  under  bulbar  symptoms  only  rarely 
occurs.  Anatomically  there  are  inflammatory  degenerative  lesions  in  the  anterior  horns  of  the 
spinal  cord.  In  contradistinction  to  spinal  muscular  atrophy,  in  chronic  poliomyelitis  the 
paralysis  precedes  the  muscular  wasting.  Further,  it  always  begins  in  the  lower  extremities 
and  runs  a  more  rapid  course.  Its  therapy  is  mainly  an  electric  one;  this  corresponds  to  the 
electrotherapy  of  infantile  spinal  paralysis. 


INFECTIOUS  DISEASES  OF  CENTRAL   NERVOUS  SYSTEM     2+7 

through  longer  duration  ( 1  to  2  weeks)  of  the  acute  stage,  through,  as  a 
rule,  greater  extent  of  the  sudden  flaccid  paralyses  and  through  slight  tendency 
to  regression.  On  the  other  hand,  corresponding  to  its  occurrence  in  com- 
pletely developed  individuals  (usually  in  the  third  decade)  the  trophic  dis- 
turbances and  the  tendency  to  contractures  are  less  marked.  From  acute  mye- 
litis this  acute  poliomyelitis  is  distinguished  by  the  absence  of  sensory  and 
sphincter  disturbances — ataxia,  spastic  symptoms,  Babinski  reflex,  etc.  (see 
Lecture  IX). 

Treatment 

In  the  infectious  stage  of  poliomyelitis  acuta  anterior,  there  is  little  to 
be  done  therapeutically,  indeed,  meddlesome  interference  is  inadvisable.  It  ap- 
pears to  me  much  more  correct  to  secure  for  the  affected  children  as  com- 
plete bodily  rest  as  possible,  than  to  disturb  them  by  frequent  warm  baths  and 
hot  applications,  as  is  the  custom  in  America,  for  example.  These  procedures 
are  intended  to  relieve  the  congestion  of  the  spinal  cord;  this  can  be  accom- 
plish! d,  however,  by  the  application  of  several  leeches  along  the  vertebral 
column,  by  mustard  paste  to  the  calves,  as  well  as  by  stimulating  the  function 
of  the  intestines.  Among  purgatives,  calomel  is  preferred  with  the  idea  that 
it  exerts  an  antiseptic  action.  Upon  the  same  ground  quinine  salol,  aspirin, 
etc.,  are  recommended.  I  must,  however,  confess  a  great  scepticism  as  to  all 
these  remedies.  Only  after  the  patient  has  entered  the  stage  of  paralysis  can 
we  interfere  with  somewhat  more  confidence  in  order  to  aid  the  reparative 
processes.  Here  strychnin  is  the  medication  of  choice,  best  in  the  form  of 
daily  subcutaneous  injections  which,  of  course,  must  be  carefully  adapted  to 
the  age  of  the  children.  For  example,  in  the  second  year  of  life,  in  which 
the  disease  occurs  most  frequently,  0.0005  (gr.  K20))  given  in  one  dose  during 
the  day.  Further,  favoring  diaphoresis  with  hot  infusions  (for  instance,  lime 
Bower  tea),  hot  packs,  hot  air  apparatus  ("Phoenix,"  etc.)  are  to  be  recom- 
mended. On  the  other  hand,  too  early  beginning  electrical  and  massage  treat- 
ment is  quite  properly  opposed  by  both  neurologists  and  pediatrists.  It  is 
hist  to  hold  to  Opperihevm's  rule  not  to  begin  until  after  two  or  three  weeks 
have  elapsed,  while  at  the  start  the  patient  is  kept  continuously  in  bed,  which 
restriction  is  only  to  be  relaxed  later  in  the  stage  of  regression.  When  we 
have  once  begun  with  electricity  and  massage,  this  treatment  must  be  kept 
up  for  a  long  period,  until  definite  entry  upon  a  stationary  condition  is  no 
longer  dubious,  on  an  average,  for  about,  a  year.  The  elect  ro-therapeut  ic 
methods  here  applicable  have  already  been  described  in  Lecture  III  and 
Lecture  VII,  namely,  galvanization  and  faradization  of  the  nerves  and  mus- 
cles in  the  paralyzed  regions,  «nd  the  galvanic  current  passed  through  the 
spinal  cord.  One  must  begin  with  very  weak  currents  and  gradually  accustom 
the  little  patient  to  stronger  ones.  .Massage,  also,  must  be  carried  out  in  a 
mild,  careful  manner,  and  it  must  he  attempted  to  stretch  the  muscles  which  are 
exposed  to  contracture  as  well  as  to  preserve  the  mobility  of  the  different 
joints.  Vibration  massage  of  the  articulations  tends  to  counteract  trophic 
disturbances   of   the   capsule   atid   ligaments.      The   carrying  out   of  active   gym 

nastic  exercises   is  also  important:  those  movements  which   the  children  may 


248  LECTURE    XVI 

little  by  little  become  able  to  practice  again,  must  (systematically  with  avoid- 
ance of  all  overexertion)  be  carried  out,  and  against  regulated  increasing  re- 
sistance. Massage  and  exercise  therapy  combats  sluggishness  of  circulation, 
and  so  brings  the  muscles,  integument  and  skeleton  under  better  conditions  of 
nutrition.  The  very  popular  salt-bath  cures,  and  rubbing  with  liniments 
which  stimulate  the  skin  (linimentum  ammonias,  linimentum  saponis,  spiritus 
juniperi,  etc.),  accomplish  the  same  thing.  Of  modern  procedures,  "diather- 
my," after  Nagelschmidt,  Bergonie,  etc.,  appears  to  act  favorably.  In  no 
case  should  it  be  neglected  to  reduce  conditions  favoring  the  production  of 
deformities  as  much  as  possible,  by  the  application  of  suitable  splints,  bed- 
rests, etc.  If  the  patient  has  entered  the  stage  of  trophic  disturbances  and 
contractures,  he  should  come  under  the  care  of  the  orthopedist.  This  is  not 
the  place  to  discuss  at  length  the  often  very  successful  operations  (arthrode- 
sis, transplantations,  plastic  operations  on  tendons,  etc.)  or  the  various  ortho- 
pedic apparatus,  as  they  have  been  contrived  and  introduced  by  Vulpius, 
Hoffa,  Nicoladoni,  Hiibscher,  Schvlihess,  Hessing,  and  others. 

B.     Epidemic  Cerebro-Spinal  Meningitis 

In  contradistinction  to  epidemic  infantile  paralysis,  epidemic  meningitis  is 
a  bacteriologically  well  characterized,  acute  infectious  disease.  It  is  to-day 
established  beyond  a  doubt  that  the  meningococcus  intracellularis  (discovered 
by  Weichselbaum  in  1887)  is  its  specific  cause.  This  is  a  micro-organism,  in 
its  biscuit  shape  and  its  position  within  the  leucocytes  resembling  the  gono- 
coccus  which,  inoculated  into  dogs,  monkeys,  or  goats,  produces  a  meningitis. 
Nevertheless,  mixed  infections  with  other  pathogenic  germs,  particularly  with 
the  pneumonococcus  and  the  diplococcus  crassus,  occur.  Epidemics  of  the  last 
sort  have  been  observed  particularly  in  barracks.  The  method  of  infection 
is  connected  with  living  in  close  association  and  occurs  (since  the  meningo- 
coccus may  remain  viable  in  the  naso-pharyngeal  mucus  even  of  healthy  indi- 
viduals, for  months)  regularly  by  "drop  infection"  in  coughing,  hawking, 
sneezing.  Westenhoffe%  has  recognized  that  its  passage  into  the  meninges 
takes  place  from  the  pharyngeal  tonsil  and  the  lymphatic  tissue.  Children 
with  hyperplasia  of  these  structures  should  be  considered  as  predisposed. 
Ordinary  anginas  favor  the  infection,  hence  the  preponderant  occurrence  of 
epidemics  in  winter,  and  in  the  cold,  damp  spring  months.  That  traumatic 
influences  which  affect  the  skull  (in  children,  sometimes  boxes  on  the  ear  from 
the  teacher  or  from  the  parent,  have  been  held  responsible  for  the  outbreak 
of  meningitis),  are  to  be  considered  as  exciting  causes,  is  not  certainly  proved, 
but  quite  plausible.  Besides  the  lymph  channels  the  blood  vessels  are  also 
open  to  invasion  of  the  meningococcus,  as  culture  experiments  after  punctur- 
ing the  vein  have  shown.  The  first  meningitis  epidemic  was  observed  in  our 
country  (Geneva)   in  1805. 

The  pathological  anatomy  of  the  affection,  apart  from  inconstant  myo- 
carditic  changes  and  rather  regular  inflammatory  lesions  of  the  tonsils  and  the 
middle  ear,  consists  predominantly  of  meningitic  processes.  The  brain  mem- 
branes, particularly  their  basal  region,  usually  are  much  more  decidedly  affected 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     2-19 

than  the  membranes  of  the  cord.  There  is  dependent  upon  the  intensity  of  the 
case,  and  upon  the  stage  in  which  it  comes  to  autopsy,  formation  of  a  serous, 
sero-fibrinous  or  fibrino-purulent  exudate  between  the  pia  mater  and  the 
arachnoid.  The  inflammation,  however,  also  extends  along  the  vessels  or  the 
nerve  roots  to  the  central  organ  itself.  In  the  brain,  abscesses,  porencephalic 
defects,  inflammatory  hydrocephalus,  etc.,  can  occur. 

Symptomatology  and  Course 

Epidemic  cerebrospinal  meningitis  usually  seizes  its  victims  brutally  with- 
out prodromal  symptoms,  with  sudden  rise  of  temperature  and  repeated  chills. 
In  young  children  convulsions  are  not  very  rarely  observed  as  an  initial  symp- 
tom. Only  in  the  minority  of  cases  a  more  or  less  marked  feeling  of  illness, 
eventually  with  stupor  and  vomiting,  precedes  the  disease  proper.  This,  as  a 
rule,  in  from  2-i  to  -18  hours  after  the  onset  of  the  fever,  has  readied  its  full 
height,  and  then  presents  the  following  symptoms  in  more  or  less  intense 
degree : 

Headache  of  great  violence  and  persistence,  which  appears  to  have  its 
maximum  intensity  in  the  occipital  region ;  very  frequently,  also,  the  vertebral 
column  is  the  seat  of  spontaneous  pains,  particularly  in  the  cervical  and  lum- 
bar regions.  Both  the  occipital  region  and  the  spinous  processes  of  the 
vertebral  column  show  great  sensitiveness  to  percussion  and  pressure.  Besides 
this,  there  is  general  hyperesthesia,  all  handling  of  the  patient  elicits  expres- 
sions of  pain ;  there  is  also  great  sensitiveness  to  optic  and  auditory  stimuli. 
Leichenstern' s  phenomenon  seems  to  indicate  hyperesthesia  of  the  skeleton; 
the  patient  when  tapped  on  the  bones  of  one  of  his  extremities  draws  himself 
up  violently,  often  with  a  cry.  The  "hydrocephalic  cry"  of  Trousseau,  a  shrill 
scream  which  some  patients  even  in  deep  stupor  emit  from  time  to  time,  is 
perhaps  also  to  be  considered  as  an  expression  of  pain. 

The  "stiff  neck"  which  has  given  the  disease  its  popular  name  *  imposes 
itself  as  pathognomonic  even  on  the  laity.  Even  on  slight  development  of  this 
dreaded  symptom  it  is  noticeable  that  the  patient  does  not,  as  is  generally 
the  rule  in  other  severe  illnesses,  let  the  chin  sink  more  or  less  upon  the  chest, 
but  that  he  deflects  the  head,  and  the  occiput  bores  into  the  pillow.  Active 
as  well  as  passive  movement  of  the  neck  is  hindered  in  an  increasing  degree; 
our  attempts  to  bend  his  head  forward  are  answered  by  the  patient  with  ex- 
pressions of  severe  pain.  Later,  the  rest  of  the  vertebral  column  becomes 
stiff  as  a  board.  This  high  degree  of  hypertonia  extends  farther  to  the  mus- 
cles of  the  extremities:  in  the  lower  extremities  it  is  accompanied  by  a  draw- 
ing them  up  on  the  body  (tendency  to  contracture  in  flexion)  through  which 
can  be  produced  the  anomaly  "I'  position  which  has  been  called  by  the  French 
on  account  of  its  analogy  to  the  shape  of  a  gun-flint,  "attitude  en  chien  de 
fusil."  Here  belongs  also  Kermg's  sign,  which  depends  upon  the  impossibility 
of  bringing  down  the  extremities  of  the  patient  placed  in  a  sitting  posture  to 
the  plane  of  the  bed  from  their  position  of  flexion  at   the  hip  and  knee,      further 

*  German  "Gcnickstnrrc." 


250  LECTURE    XVI 

phenomena  characteristic  of  meningitis  arc  masklike  rigidity  of  the  counte- 
nance, sometimes  a  moderate  degree  of  trismus,  with  audible  grinding  of  the 
teeth,  particularly  frequent,  however,  the  "boatlike"  drawing  in  of  the  abdom- 
inal walls. 

The  tendon  and  skin  reflexes  are  usually  exaggerated,  their  loss  is  an 
ominous  symptom.  Vasomotor  irritability  is  also  increased,  from  which  the 
particular  form  of  dermographia  (see  Lectures  XXIII  and  XXVII)  can  ap- 
pear, which  Trousseau  denominated  "tache  cerebrale."  Stroking  the  integu- 
ment leaves  behind  Maine-red  streaks  which  only  pale  after  considerable  time. 
A  further  cutaneous  symptom  of  epidemic  spinal  meningitis  is  herpes,  which 
in  the  majority  of  cases  appears  on  the  lips  and  face  between  the  second  and 
sixth  day  of  the  disease,  while  scarlatiniform  erythemata,  multiple  hemor- 
rhages into  the  skin,  or  a  roseola  resembling  that  of  typhoid  fever  are  quite 
atypical  findings. 

Further  symptoms  of  the  disease  are  vomiting,  stupor,  less  frequently  de- 
lirium, further,  retention  of  urine  and  stools  or  ischuria  paradoxa  (dribbling 
of  the  urine  when  the  filling  of  the  bladder  has  reached  a  certain  degree), 
often  rapid  emaciation,  occasionally  albuminuria  and  glycosuria.  Character- 
istic of  the  fever  is  a  continuous  course  at  the  start  with  temperatures  between 
39°  and  40°  C,  which  later  a  stage  of  alternating  remissions  and  exacerbations 
follows ;  its  rise  to  a  hyperpyretic  degree  is  prognostically  bad  and  often  im- 
mediately precedes  death.  In  cases  which  enter  upon  recovery,  the  fever 
disappears  by  lysis.  As  to  the  pulse-rate,  it  should  be  emphasized  that  it  is 
relatively  low  compared  to  the  temperature,  as  a  rule  (for  example,  100  for 
40°,  80  for  39°),  and  that  in  convalescence  a  slow  pulse  is  the  rule  (.50  and 
lower).  A  rapid  and  considerable  increase  in  frequency  during  the  disease 
points  to  threatened  exitus. 

As  to  whether  the  slow  pulse  should  be  considered  a  vagus  symptom,  views 
differ.  On  the  other  hand,  other  cranial  nerves  in  many  cases  are  affected  in 
unmistakable  manner,  particularly  the  optic  and  the  auditory.  While  the 
meningitic  optic  affection  (which  shows  itself  ophthahnoscopically  as  papil- 
litis), in  cases  which  survive,  seldom  leaves  behind  amaurosis,  auditory  neuritis 
usually  causes  impaired  hearing,  sometimes,  indeed,  deafness.  The  pupils 
are  usually  narrow  and  react  sluggishly  for  light  and  accommodation;  on  the 
other  hand,  they  often  dilate  markedly  when  the  integument  is  irritated.  This 
last  symptom,  described  by  Goppert,  can  be  obtained  by  stroking  the  skin 
with  a  finger-nail.  Paralyses  of  the  facial  or  of  the  external  eye  muscles 
are   rarer. 

In  a  limited  number  of  the  cases,  pus  collections  in  the  eye,  in  the  middle 
ear,  in  the  brain  substance  occur,  partly  by  direct  conduction,  in  part  by 
metastasis.  With  the  last,  there  are  produced  spasms  of  constant  localization 
(for  example,  in  one  side  of  the  face,  in  one  arm,  etc.),  which  leave  behind  a 
paralysis  of  the  muscles  affected.  Also  a  meningococcic  endocarditis  occurs. 
In  the  blood  there  is  regularly  a  hyperlcucocytosis ;  according  to  Goppert 
and  others,  high  lcucocytosis  (over  24,000)  is  prognostically  serious.  It  can 
reach  60,000.  As  long  as  the  lcucocytosis  is  not  diminishing  new  exacerba- 
tions may  be  expected,   even   when   the  disease   picture   otherwise  has   consid- 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     251 

erably  improved.  Finally,  at  the  height  of  the  disease  a  considerable  poly- 
nucleosis shows  itself  in  the  spinal  fluid  (see  Lecture  XII,  page  187).  In  the 
stage  of  convalescence  (according  to  Acharil  and  others)  the  polynuclear 
leucocytes  are  replaced  by  numerous  lymphocytes.  Bacteriologicallv  the  me- 
ningococcus can  be  recognized  in  the  cerebro-spinal  fluid,  as  Heubner  first 
showed. 

According  to  the  course  of  epidemic  cerebro-spinal  meningitis,  different 
forms  of  it  have  been  distinguished.  In  the  "foudroyant"  form  (meningitis 
eerebro-spinalis  siderans)  the  patients  die  within  a  few  hours  after  the  begin- 
ning of  the  first  symptoms;  indeed,  they  can  after  relatively  slight  prodromes 
(slight  fever,  headache,  nausea,  etc.)  with  a  loud  scream  fall  dead  to  the 
ground,  in  which  case  we  speak  of  apoplectiform  meningitis.  In  a  few  cases 
of  the  "foudroyant"  form  there  has  been  no  rise  of  temperature.  The  rudi- 
mentary or  abortive  cases,  in  which  the  chief  symptoms  of  the  disease  are  only 
present  in  an  undeveloped  manner,  form  the  other  extreme.  There  is  slight 
fever,  some  headache  and  nausea,  feeling  of  tension  in  the  neck  and  in  the 
limbs,  sensitiveness  to  touch,  noises,  etc.  Such  cases  recover  in  from  8  to 
14  days:  when  they  appear  sporadically,  they  are  scarcely  ever  diagnosed, 
but  their  connection  with  cerebrospinal  meningitis  is  only  recognized  during 
epidemics.  In  the  acute  forms,  however,  death  in  coma  occurs  in  from  1  to  3 
weeks  after  the  outbreak  of  the  disease.  Finally,  there  is  a  prolonged,  remit- 
tent; or  even  intermittent  form,  which  with  alternating  improvement  and  re- 
lapse, can  extend  over  weeks  or  even  months.  Termination  in  recovery  is 
possible;  usually,  however,  death  in  a  so-called  "status  hydrocephalicus"  finally 
occurs,  after  the  most  extreme  emaciation  and  exhaustion,  tonic  contractures 
and  flexion  of  the  extremities,  deep  coma,  frequent  vomiting,  etc.  In  conva- 
lescence after  epidemic  cerebrospinal  meningitis,  the  danger  of  relapse  is 
always  great. 

Prognosis 

After  what  has  been  said  above,  the  prognosis  of  cerebrospinal  menin- 
gitis is  naturally  a  very  grave  one.  The  mortality  varies  from  epidemic  to 
epidemic  within  comparatively  wide  limits  (30  to  70  per  cent.)  ;  as  an  average 
it  is  about  40  to  50  per  cent.  The  more  violent  the  commencement,  the  iess 
tin  hope  of  survival.  Even  among  the  so-called  "recovered"  a  large  number 
remain  severely  injured  for  life  (blindness,  partial  or  complete  deafness,  mental 
reduction).  Others  escape  with  less  impairment  (tinnitus  aurium,  squint, 
tendency   to   headaches   or  neuralgias). 

Treatment 

From  Hie  point  of  view  of  prophylaxis,  isolation  of  the  patients,  accord- 
ing to  the  general  rules  in  infectious  diseases,  is  to  he  ordered.  The  isolation 
■{"carriers,"  that  is,  healthy  pel-sons  who  have  meningococci  in  their  naso- 
pharyngeal secretions  and  who  contribute  to  spreading  the  epidemic,  is  unfor- 
tunately only  exceptionally  attainable  (for  example,  in  epidemics  in  barracks). 


252  LECTURE    XVI 

In  general  one  must  content  himself  with  having  the  relatives  of  the  patient 
use  disinfecting  washes,  irrigations,  etc.,  for  careful  cleansing  of  the  naso- 
pharyngeal space.  If  a  serum  prepared  by  Wassermawn  and  Kollc  for  this 
purpose  is  specially  efficacious,  we  must  wait  and  see;  favorable  results  have 
been  obtained  from  it  by  Kutscher. 

Also  with  regard  to  the  result  of  specific  treatment  with  meningococcus- 
sera  (such  have  been  prepared  by  Jochmann,  Flexner,  Kolle,  Wasxermann,  and 
others,  positive  statements  are  not  yet  possible,  since  in  consequence  of  the 
very  variable  malignity  of  different  epidemics  (see  above,  page  251)  the  com- 
parison between  the  mortality  in  cases  in  which  the  sero-therapy  was  carried 
out  and  those  in  which  it  was  not  gives  too  great  room  for  subjective  impres- 
sions. Nevertheless,  it  is  imperatively  necessary  to  clear  up  the  subject  by 
further  researches.  According  to  Peritz  the  following  rules  should  regulate 
such  investigations: 

If  on  the  first  lumbar  puncture  in  a  suspicious  case,  the  cerebrospinal 
fluid  is  tin  bid.  serum  (it  should  not  be  older  than  three  months)  should  be  at 
once  injected,  and  only  when  the  fluid  is  clear  should  we  wait  for  bacteriolog- 
ical examinations.  When  the  puncture  is  made,  as  much  fluid  as  possible  is 
withdrawn,  and  then  30  cc  of  the  serum  is  injected  into  the  subarachnoid  space, 
even  when  less  fluid  than  this  has  been  withdrawn.  When  a  larger  quantity 
of  fluid  has  escaped,  as  much  as  45  cc  of  the  serum  can  be  given.  In  very 
severe  cases  this  last  is  particularly  desirable.  An  appreciable  resistance  to-; 
the  injection  gives  a  signal  for  its  cessation  (Levi/  recommends  limiting  the 
first  injection  to  10  cc  in  small  children  and  to  20  cc  in  adults).  In  malignant 
cases,  if  there  is  no  improvement  the  injection  should  be  repeated  in  twelve 
hours.  Except  in  the  very  mildest  cases  the  injections  should  be  repeated 
daily  for  four  days.  If  then  diplococci  are  still  found  in  the  spinal  fluid,  the 
injections  should  be  continued;  when,  however,  after  disappearance  of  diplo- 
cocci from  the  fluid  and  in  spite  of  four  injections,  the  subjective  symptoms, 
including  the  fever  and  stupor,  continue,  Ave  should  wait  four  days,  and  then 
if  there  is  no  improvement  repeat  the  four  injections.  Each  exacerbation 
should  naturally  be  treated  on  the  same  day.  Each  time  the  process  flares  up 
again,  we  should  at  once  begin  the  four-day  treatment  and  proceed  otherwise 
as  on  the  first  occasion.  This  plan  of  treatment  is  to  be  continued  until  the 
patient  is  free  from  symptoms,  the  diplococci  have  disappeared  from  the 
spinal  fluid,  or  the  disease  has  entered  upon  the  chronic  stage. 

As  you  see,  this  procedure  demands  numerous  lumbar  punctures  and  that 
these  last  by  themselves,  that  is,  even  without  scrum  therapy,  present  a  rational 
method  of  treatment — corresponding  to  the  rule  "where  there  is  pus,  evacu- 
ate"— is  evident  without  further  discussion.  They  are  undertaken  then  quite 
generally  in  epidemic  meningitis,  and  usually  as  a  routine  procedure,  that  is, 
daily  or  every  second  day,  10  to  20  cc  of  fluid  are  removed.  A  few  authors 
have,  indeed,  no  hesitation  in  removing  sometimes  far  greater  quantities  of 
fluid  Zupnik,  for  example,  70  to  90  cc.  The  headache  and  stupor  often 
markedly  decrease  after  this  procedure. 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     253 


TECHNIQUE    OF    LUMBAR    PUNCTURE 

The  puncture  of  the  subarachnoid  space,  first  done  by  the  American  neu- 
rologist Leonard  Corning  and  soon  after  introduced  into  clinical  practice  by 
Quincke,  is  made  in  the  lower  portion  of  the  lumbar  region  of  the  vertebral 
column.  This  localization  suggests  itself  from  the  necessity  of  avoiding  injury 
of  the  spinal  cord  with  the  puncture  needle ;  the  cord,  however,  does  not  extend 
below  the  second  lumbar  vertebra.  Further  distallv,  indeed,  the  "cauda  equina" 
lies  in  the  subarachnoidal  space,  but  the  nerves  composing  it  are  always  pushed 
aside  by  the  needle  so  that  appreciable  wounding  of  them  does  not  occur. 
Slight  pricking  of  them  produces  onty  a  short,  lightninglike  pain.     We  punc- 


Fio.  76. 
Anatomical  Points,  tor  Guidance  in  Making  a  Lumbar  Puncture. 


ture  usually  between  tin-  4th  and  5th  vertebrae,  though  the  puncture  can  be 
made  between  the  2d  and  .'id  or  between  the  5th  lumbar  vertebra  and  the 
lacrum.  Topographic  orientation  is  very  easy,  .since  a  line  drawn  through  the* 
highest  points  of  the  ilia  passes  over  Hie  point  of  the  spinous  process  of  the 
4th  lumbar  vertebra  (sec  Fig.  70).  We  use  a  hollow  needle,  at  least  S  cm 
ping  and  <>(  1  mm  externa]  caliber  and  <).(>  nun  lumen,  shortly  but  sharply 
beveled  al  Hie  point;  a  mandrill  should  be  inserted  during  the  puncture.  This 
cm  he  made  in  the  median  line-in  the  space  between  the  4th  and  the  5th 
lumbar  vertebra?;  it  is  in  general  better,  however,  to  make  the  puncture  about 
1  cm  to  the  right  of  the  middle  line,  since  by  so  doing,  on  the  one  hand  the 
lough  interspinous  ligament  is  avoided,  and  on  the  other  the  needle  enters 
the  somewhat  more  roomy  lateral  part  of  the  intervertebral  foramen.  Of 
course,  such  a  ••paramedian"  puncture  demands  that  the  needle  he  directed 
■Omewhat  toward  the  median  line,  as  shown  in  Fig.  77.  for  the  rest,  in 
children     the    needle    should    be    introduced     al     Hie     ideal    cross  sect  ion    of    the 


254 


LECTURE    XVI 


trunk;  in  adults  it  must  usually  be  introduced  somewhat  more  frontallv. 
The  operation  is  further  made  easier  by  strong  flexion  of  the  vertebral  column, 
since  by  this  the  space  between  the  arches  of  the  vertebrae  is  enlarged.  Pa- 
tients who  can  sit  up  must  hence  bring  their  heads  forward  as  low  as  possible; 
where  this  is  not  possible,  as  is  almost  always  the  case  in  meningitis,  the 
patient  is  laid  on  his  side  and  his  knees  bent  up  as  far  as  possible.  The  skin 
over  the  point  of  entrance  is  disinfected  by  painting  it  with  fresh  tincture  of 
iodine,  and  eventually  is  anesthetized  with  the  chloride  of  ethvl  spray.  The 
hands  are  carefully  disinfected  (by  soap,  scrubbing  and  alcohol),  and  the 
previously  boiled  needle  is  introduced  in  the  direction  indicated.  The  ligamen- 
tum  flavum,  which  is  stretched  between  the  arches  of  the  vertebra?,  gives  an 
elastic  resistance  which,  however,  suddenly  yields,  and  the  point  of  the  needle 
enters  the   subarachnoid   space.      If,   however,   the   needle  is   wrongly  directed 


ltroduction  of  the  Needle  in  Lumbar  I'tincture. 


and  strikes  the  hone  instead  of  the  yellow  ligament,  it  is  somewhat  withdrawn 
and  directed  either  more  frontally  or  more  caudallv.  If  the  instrument  has 
been  properly  introduced,  the  mandrill  is  withdrawn  and  the  fluid  escapes  in 
drops  or,  when  the  pressure  is  increased,  in  a  stream.  If  the  needle  gets 
stopped  up  by  coagulum,  which  is  not  rare,  the  obstruction  is  to  be  removed 
by  reintroduction  of  the  mandrin.  If,  instead  of  fluid,  blood  appears,  the 
needle  has  probably  entered  a  vessel  of  the  venous  plexus  which  covers  the 
posterior  aspect  of  the  body  of  the  vertebra ;  in  this  case  the  needle  is  with- 
drawn several  mm  until  no  more  blood  escapes.  That  anomalies  of  the  verte- 
bral column  or  adhesions  of  the  arachnoidal  sac  render  lumbar  puncture 
impossible  is  a  rare  happening.  The  depth  to  which  the  needle  must  penetrate 
varies  from  2  cm  in  small  children  and  6  or  7  cm  in  adults.  After  lumbar 
puncture  the  patient  should  remain  in  a  horizontal  position  for  at  least  24 
hours. 

Bier,  Vorschiitz  and  Eckert  have  recommended  the  combination  of  lumbar 
puncture  with  "stasis."     Two  hours  after  the  puncture  an  elastic  band  is  placed 


INFECTIOUS  DISEASES  OF  CENTRAL  NERVOUS  SYSTEM     255 

about  the  neck,  tight  enough  to  produce  a  slight   cyanosis  of  the  face,   and 
kept  on  about  20  hours. 

In  general  use  are  hot  baths — which,  according  to  Heubner's  directions, 
should  be  given  daily — in  which  beginning  with  35°  C,  the  temperature  should 
be  raised  slowly  to  40°  and  more.  On  the  other  hand,  cold  baths  and  affusions, 
as  are  customary  in  typhoid,  are  recommended.  Application  of  the  ice-bag 
or  of  the  cold  cap  to  the  head  and  along  the  spine  usually  reduce  the  headache. 
Further,  derivative  procedures,  as  leeches  or  cupping  over  the  mastoid  and 
along  the  vertebral  column,  inunction  of  gray  ointment  over  the  neck,  blisters, 
frequent  purgation  (calomel),  mustard  paste  to  the  calves,  etc.,  can  be  used. 
Of  drugs,  antipyretics  and  sedatives  come  into  consideration  (salicylate  of 
sodium,  antipvrin,  pyramidon,  chloral,  morphin,  etc.).  More  important,  how- 
ever, is  the  carrying  out  of  the  difficult  and  responsible  nursing;  namely,  by 
the  choice  of  concentrated  food,  its  frequent  introduction  (when  it  is  necessary 
with  the  oesophageal  tube),  the  rapid  loss  of  strength  is  to  be  combated  as 
much  as  possible.  In  convalescents  the  most  careful  oversight  and  tonic 
treatment  is  in  place  (fresh  air,  sea  and  mineral  baths,  arsenic,  strychnine,  etc.). 

Xote. — On  account  of  the  discovery  that  when  hexamethylamin  (urotropin)  is  given  hy  the 
month  it  appears  in  the  spinal  fluid,  this  drug  is  quite  generally  administered  in  infectious  dis- 
eases of  the  central  nervous  system,  particularly  in  poliomyelitis  and  cerebrospinal  meningitis, 
its  value  is,  however,  uncertain.— 7Y««W<(/ui\ 


LECTURE  XVII 
Encephalorrhagia  and  Encephalomalacia 

(Cerebral  Hemorrhage  and  Cerebral  Softening) 

Gentlemen  :  We  turn  now  to  the  exceedingly  important  disease  pictures 
which  arise  from  more  or  less  extended,  circumscribed  destructions  of  the 
brain  substance  upon  a  basis  of  vascular  lesions.  Now  it  is  rupture  of  an 
artery,  again  occlusion  of  an  artery  (from  endarteritis,  thrombosis  or  em- 
bolism). In  the  first  case  a  pouring  out  of  blood  destroys  the  cerebral  pa- 
renchyma, in  the  last  this  undergoes  ischemic  softening.  Now,  before  we 
consider  separately  encephalorrhagia  (cerebral  hemorrhage)  and  encephaloma- 
lacia, we  will  take  up  the  most  typical  of  the  symptom  complexes  through 
which  they  manifest  themselves  clinically — cerebral  hemiplegia.  Since  now, 
however,  of  these  cerebral  hemiplegias  by  far  the  greatest  number  have  their 
focus  in  the  internal  capsule,  our  description  of  the  semiology  will  be  based 
upon  this  form. 

Capsular  Hemiplegia 

This  is,  as  a  rule,  the  result  of  a  cerebral  hemorrhage.  That  the  internal 
capsule  is  the  favorite  seat  of  this  last  is  due  to  the  anatomical  relations  of 
the  vascular  distribution.  The  middle  cerebral  artery,  the  most  important 
branch  of  the  internal  carotid,  gives  off  on  the  base  of  the  brain,  branches 
mounting  vertically  to  the  optic  thalamus,  the  corpus  striatum,  and  the  internal 
capsule,  the  lenticulo-optic  artery,  and  the  lenticulo-striatc  arteries.  One  of 
these  last  which  passes  along  the  surface  of  the  lenticular  nucleus  to  the  internal 
capsule,  perforates  this  and  finally  ends  in  the  corpus  striatum,  has  been  given 
by  Charcot  the  name  "artery  of  cerebral  hemorrhage"  (see  Fig.  78).  These 
perforating  basal  vessels  (in  contradistinction  to  the  arteries  of  the  cortex 
which  anastomose  with  one  another)  are  so-called  "end-arteries";  since,  be- 
sides this,  they  come  off  at  nearly  right  angles  from  the  largest  branch  of  the 
carotid,  there  is  in  them  a  pressure  nearly  equal  to  that  in  the  carotid,  and 
every  increase  of  this  pressure  is  transferred  directly  to  them,  but  not  to  other 
cerebral  vessels  of  similar  caliber.  Mendel  has  been  able  to  bring  these  rela- 
tions to  demonstration  manometrically  in  a  model  of  the  cerebral  vascular 
system  made  out  of  rubber  tubes.  Variations  of  pressure  are  now,  as  we  will 
later  see,  of  decisive  importance  in  the  production  of  cerebral  hemorrhages. 

There  lies,  however,  in  the  posterior  limb  of  the  internal  capsule  nearly 
the  whole  of  the  motor  tract  for  the  opposite  side  of  the  body  together  in  a 

256 


ENCEPHALORRHAGIA    AND    ENCEPHALOMALACIA 


25't 


relatively  very  small  space  (see  Fig.  79).  Complete  hemiplegia,  hence,  is  in 
by  far  the  majority  of  cases  the  consequence  of  a  lesion  of  this  posterior  limb 
of  the  internal  capsule,  that  is,  there  is  a  cross  paralysis  of  the  lower  facial 
(the  upper  escapes  in  consequence  of  its  bilateral  representation  in  the  cortex) 
of  the  hypoglossus,  of  the  arm  and  of  the  leg.  If  the  disease  focus,  however, 
extends  also  to  the  posterior  third  of  the  posterior  limb  of  the  internal  capsule, 


Fig.  78. 
Arterial  Supply  of  the  Cerebrum  and  the  Basal  Ganglia. 

H   =Distribution  of  the  Anterior  Cerebral  Arterv. 


HD 


Distribution  of  t lie-  Middle  Cerebral  Arte 


II  =  Distribution  of  the  Posterior  Cerebral  Artery. 

C.i.  =  Internal    ('anil  id. 

A.e.ni.       Middle  Cerebral  Arterj  . 

]  =  I-enticulo  Optic  Artery. 

.'  and   :'.        Lentieulo  Striate    Arteries. 

8= So-called  "Artery  of  Cerebral  Hemorrhage." 


where  the  sensory  tracts  lie  together  immediately  after  their  exit  from  the 
pptic  thalamus,  I  here  results  besides  a  crossed  hemianesthesia  of  the  whole 
body.  Sometimes  the  destructive  focus  extends  even  to  the  posterior  end  of 
the  internal  capsule  to  the  so  called  "Carrefour  sensitif"  (sensitive  crossway), 
where  two  important  sensory  tracts,  the  optic  and  the  auditory,  branch  off, 
from  the  total  contingent  of  sensory  tracts  to  proceed  to  the  visual  and  audi 
fcory  centers  of  the  brain  cortex  (see  Fig.  ?!>)• 

In  such  cases,  now,  there  occur  also  hemianopsia  and  unilateral  deafness, 


258 


LECTURE    XVII 


both  on  the  opposite  side;  since  each  visual  area  receives  the  visual  impressions 
from  the  opposite  halves  of  the  visual  field  of  both  eyes,  as  is  shown  in  Fig.  80, 
and  each  auditory  cortical  area  the  end  neurons  of  the  auditory  tract  from 
the  opposite  ear. 

Apart   from   the  eventual   sensible   and   sensory   accompanying  symptoms, 
capsular  hemiplegias  present  also  the  following  peculiarities: 


Y  t 


Fig.  79. 
The   Internal   Capsule  and   Corona    Radiata. 


T  =  Optic  Thalamus. 

L  =  Lenticular  Nucleus. 

C  =  Caudate  Nucleus. 

F  =  Supranuclear  tract  for  the  Facial. 

H  =  Supranuclear  tract  for  the  Hypo- 
glossal. 

A  =  Supranuclear  tract  for  the  Arm 
Muscles. 

B  =  Supranuclear  tract  for  the  Leg 
Muscles. 

S  =  Sensory  tract  (Tractus  Thalamo- 
cortical). 


a  =  Auditory  tract  to  the  Temporal  Lobe. 

v  =  Visual  (Oratiolet's)  tract  to  the  Oc- 
cipital   Lobe. 

1  =  Fronto-Pontine  tract  and  fibers  to 
Thalamus. 

•2  =  Occipito-Temporo- Pontine  tract  and 
fibers  to  Thalamus. 

Balken  =  Corpus  Callosum. 

Insel  =  Island  of  Reil. 

Frontal-Lappen  =  Frontal  Lobe. 

Temporal-Lappen  =  Temporal  Lobe. 

Occipital-Lappen  =  Occipital  Lobe. 


Besides  the  upper  facial  muscles  and  on  account  of  analogous  relations  of 
innervation  (connection  of  the  corresponding  nuclei  of  the  brain  axis  or  spinal 
cord  with  both  halves  of  the  cerebrum)   the  masticatory,  deglutitory,  eye  and 


EXCEPHALORRHAGIA  AND  EXCEPHALOMALACIA 


259 


trunk  muscles  remain  unaffected.  (For  the  rest,  as  to  the  upper  facial,  it  Is, 
however,  to  be  stated  that  often  there  is  slight  diminution  in  the  contractility 
of  the  frontalis  and  the  orbicularis  palpebrarum  on  the  side  opposite  to  the 
lesion;  the  eyebrows  hang  perhaps  somewhat  deeper,  or  the  eye  can  be  kept 
closed  for  a  shorter  time  than  upon  the  homolateral  half  of  the  face.)     Also, 


Fio.  80. 
Visual  Trait  am!  Pupillary  Keflex  Paths. 


Opt.  Erinnerps.  Centr.  —  Center  fur  Optic 

Memories. 
Sehrinde  =  Cortical  Visual  (enter.      • 


Gratiolet'sche  Strahlung=  Optic  Radiations. 
Primare  Sehcentren       Primary  Visual  Centej 
Gesichtsfeld       Visual  Field. 


in  complete  hemiplegia  different  groups  of  muscles  in  the  extremities  are  regu- 
larly unequally  affected.  While  power  of  movement,  little  by  little,  returns 
in  a  great  number  of  muscles,  others  do  not  usually  recover  again  (peroneal 
muscles,  flexors  of  the  knee,  extensors  of  tin-  elbow,  extensors  of  the  hand  and 
fingers,  external  rotators  of  tin-  arm,  supinators  of  tin-  forearm).  The  arm, 
as  a  rule,  is  more  affected  than   the  leg.     The  positions,  however,  which   the 


260 


LECTURE    XVII 


extremities  assume  in  consequence  of  the  preponderating  action  of  those  mus- 
cles which  regain  their  motility  are  often  later  fixed  by  contractures.  On  this 
account  the  foot  is  held  in  equinovarus  position  and  the  knee  extended  so  that 
in  walking,  the  leg  dragging,  must  be  swung  forward  in  a  lateral  arc  ("circum- 
duction," "helicopodia").  The  arm  is  fixed  in  adduction,  the  elbow,  hand  and 
fingers  in  flexion. 

These  contractures  occur  from  the  fact  that  the  anterior  horn  cells,  liber- 
ated from  the  inhibiting  influence  of  the  pyramidal  tract,  are  stimulated  in  a 


Cerebral   Hemiplegia    from   Capsular   Hemorrhage   on   the   Right   Side.     Circumduction   of  the 
Left  Leg  in  Walking  ami  Typical  Contracture  Position  of  the  Left  Ann. 


tonic  manner  by  the  impulses  entering  through  the  posterior  roots,  and  a 
summation  of  these  stimuli  is  produced.  Since,  however,  there  are  certain 
definite  muscles  which  regain  their  motility  and  therewith  (thanks  to  the 
supranuclear  hypertonic  nature  of  cerebral  hemiplegia)  are  predisposed  to 
contracture,  this  striking  regularity  must  depend  upon  relations  of  innervation 
not  yet  sufficiently  explained  anatomically.  Probably  motor  paths  from  the 
subcortical  centers  (for  example,  the  roof  of  the  mid-brain,  the  tegmentum 
and  Dciters'  nucleus)  pass  in  a  preponderating  manner  directly  into  contact 
with  the  anterior  horn  cells  of  these  groups  of  muscles,  so  that  in  the  repara- 
tory  effort  of  the  organism  it  is  not  difficult  for  them  to  obtain  again,  in  a 
roundabout  way,  a  share  of  the  cortical  innervation.     Cases  of  cerebral  hemi- 


ENCEPHALORRHAGIA    AND    ENCEPHALOMALACIA         261 

plegia  in  which  other  muscle  groups  retain  preponderance  and  develop  con- 
tractures (so  that,  for  example,  the  leg  is  fixed  in  flexion)  form  very  rare 
exceptions.  The  hypertonia  of  capsular  hemiplegia  is  accompanied  by  increase 
of  the  tendon  reflexes.  To  this  hyperreflexia  and  the  pathological  reflex  phe- 
nomena (Babinski's,  Oppcnhcims  and  the  Mendel-Becliterew  reflexes),  clonus, 
certain  associated  movements,  and  so  forth,  which  so  often  accompany  them, 
all  that  we  said  in  Lecture  All  in  connection  with  spastic  paraplegia  applies. 
In  a  great  number  of  capsular  hemiplegias  besides,  one  or  another  of  the 
following  phenomena  can  be  demonstrated:  The  patient  is  laid  upon  a  firm 
surface,  his  arms  crossed  and  his  head  partially  supported ;  if  he  is  now  re- 
quested to  raise  himself  up,  he  bends  his  leg  on  the  paralyzed  side  at  the 
hip-joint  so  that  the  heel  is  raised  from  the  supporting  plane.  The  same  asso- 
ciated movement  (the  "hip  flexion  phenomenon")  appears  when  the  patient, 
seated,  attempts  to  lie  down  again.  As  "platysma  phenomenon"  ("signe  de 
peaucier")  is  denominated  the  energetic  contraction  of  the  platysma  of  the 
healthy  side,  which  becomes  visible  when  the  cerebral  hemiplegic  opens  his 
mouth.  Finally,  an  interesting  modification  of  the  radius  reflex,  which  con- 
sists in  the  occurrence  of  a  flexion  of  the  fingers  instead  of  the  normal  flexion 
of  the  forearm  at  the  elbow,  when  the  radius  on  the  paralyzed  side  is  tapped 
at  its  distal  extremity,  may  be  mentioned.  The  skin  reflexes  (namely,  the 
abdominal  and  cremaster  reflexes),  on  the  contrary,  are  almost  always  reduced 
or  absent  on  the  paralyzed  side;  this  symptom  can  be  used  while  the  patient 
is  lying  unconscious  after  an  apoplectic  stroke,  to  determine  on  which  side 
of  the  body  the  hemiplegic  paralysis  will  be  found  after  he  comes  out  of  the 
coma.  This  phenomenon  is  explained  in  (mite  plausible  manner  as  follows: 
The  centripetal  fibers  concerned  in  the  abdominal  and  cremaster  reflexes  enter 
the  cord  through  the  posterior  roots  of  the  lower  dorsal  and  upper  lumbar 
regions  and  their  centrifugal  fibers  come  through  the  anterior  roots  from  the 
same  part  of  the  spinal  cord.  But  the  stimulating  process  is  not  transmitted 
here  through  the  direct  spinal  reflex  arc,  but  through  the  intermediary  of  in- 
terposed neurones  which  first  proceed  in  a  frontal  direction  into  the  cerebrum 
and  then  again  run  caudallv.  We  cannot  propose  any  further  hypotheses 
concerning  this,  in  any  case,  complicated  mechanism.  The  conjunctival  reflex 
is   also   often   absent   on   the   paralyzed    side. 

Capsular  hemiplegia  is  a  nondegenerative  paralysis.  Nevertheless,  there 
are  certain  exceptional  cases  in  which  iii  the  paralyzed  or  even  in  Hie  recovered 
muscles,  the  so-called  "cerebral  atrophy"  which  cannot  be  referred  to  inactivity 
and  is  accompanied  by  reduction  of  the  electric  irritability,  although  without 
reaction  of  degeneration,  develops.  With  this,  arthropathies  like  those  in 
tabes  have  been  observed.  Still  more  isolated  are  the  observations  of  atrophy 
of  bone  upon  the  paralyzed  side.  .More  frequent,  on  the  contrary,  than  all 
these  trophic  disturbances,  are  anomalies  of  the  vascular  innervation:  the 
paralyzed  parts  are  cyanotic  and  feel  cold;  sometimes  there  develops  quite 
decided    localized   (edema. 

Tin-  clinical  picture  jusl  sketched  you  need  not  expect  immediately  after 
the  occurrence  of  a  cerebral  hemorrhage  or  of  a  sudden  plugging  of  a  vessel, 
u  I  have  already  indicated  in  speaking  of  the  condition  of  the  skin  reflexes 


262  LECTURE    XVII 

Much  more  do  these  happenings  accompany  a  serious,  though  also  usually 
transitory  injury  of  the  whole  brain,  which,  since  Hippocrates,  has  been  termed 
"apoplexy"  ( aTtoizXyjxrsiv — to  strike  down).  We  will  proceed  to  the  clinical 
description  of  this  condition. 

The  Apoplectic  Attack 

The  most  striking  symptom  of  the  apoplectic  attack  is  the  sudden  loss 
of  consciousness,  which  is,  indeed,  sometimes  preceded  by  certain  warnings, 
as  feeling  of  dizziness,  paresthesias  in  the  limbs,  headache,  nausea,  darkening 
of  the  field  of  vision;  the  patient  is  restless,  begins  to  totter,  and  then  sud- 
denly falls  into  a  lifeless  heap.  He  lies  there  unconscious,  usually  with  con- 
gested and  swollen  face  and  stertorous  breathing.  There  is  incontinence  of 
urine  and  stools,  only  exceptionally  retention.  The  limbs  are  all  flaccid,  the 
tendon  reflexes  are  lost.  Nevertheless,  we  are  enabled  now  and  then  to  recog- 
nize, even  at  this  time,  which  side  of  the  brain  has  been  affected.  For  example, 
if  when  raised  up  and  then  suddenly  let  loose,  the  extremity  on  one  side  falls 
more  heavily  to  the  bed  than  the  other  and  remains  stretched  out  straight  upon 
it,  this  is  the  one  in  which  hemiplegia  will  later  be  manifested.  On  this  side, 
also,  sometimes  the  hand  brought  into  supination  falls  back  into  pronation 
much  quicker  than  on  the  other  side.  Further,  the  limbs  on  the  side  opposite 
to  the  lesion,  when  the  patient  is  exposed,  chill  more  rapidly  than  the  others. 
Also,  observation  of  the  face  may  permit  localization  of  the  disease  focus, 
since  the  sail-like  flapping  of  the  cheeks  in  expiration  in  the  comatose  patient 
(so-called  "blowing  smoke")  is  more  marked  upon  one  side  (the  paralyzed  one). 
Occasionally  it  is  remarked  also  that  the  head  and  eyes  of  the  apoplectio 
patient  are  directed  toward  one  side,  and  when  the  face  is  brought  to  the 
front  again  it  always  returns  to  this  forced  position.  This  is  Vul plan's  "con- 
jugate deviation"  ("deviation  conjuguee  de  la  tete  et  des  yeux").  This,  also, 
can  well  be  applied  in  the  localization  of  the  disease  focus,  since  the  prostrate 
and  relaxed  apoplectic  turns  his  head  and  eyes  to  the  side  of  the  body  un- 
affected by  the  hemiplegia.*  We  have  already  spoken  of  the  absence  of  the 
abdominal  and  cremaster  reflexes  on  the  hemiplegic  side  and  its  importance 
as  an  early  symptom. 

The  temperature  of  the  body  usually  falls  below  the  normal  in  the  apo- 
plectic attack,  to  rise  later  to  a  febrile  degree.  When  the  course  is  favorable 
it  returns  to  normal  after  some  variation ;  a  continued  mounting  of  the  fever 
is  very  unfavorable  prognosticallv.  It  depends  so  very  frequently  upon 
broncho-pneumonic  processes,  that  many  neurologists  would  make  trophic  dis- 
turbances in  consequ'ence  of  the  cerebral  attack  responsible  for  the  rapid 
occurrence  of  these  last.  "Acute  decubitus,"  developing  occasionally  imme- 
diately after  the  ictus  (in  the  region  of  the  sacrum),  an  ominous  sign,  encour- 
ages a  similar  hypothesis. 

The   apoplectic   coma   usually   lasts    some   hours,   but   unconsciousness    for 

*  In  contradistinction  to  this,  patients  who  show  unilateral  convulsions  from  irritative  cere- 
bral lesions,  in  the  ease  of  conjugate  deviation,  look  toward  the  convulsed  extremities. 


ENCEPHALORRHAGIA    AND    ENCEPHALOMALACIA         263 

several  days  is  nothing'  unusual.  The  longer  this  condition,  which  must  be 
considered  as  a  sort  of  concussion  of  the  brain,  lasts  the  worse  in  general  is 
the  prognosis.  It  is  particularly  unfavorable  in  cases  in  which  even  dining 
the  coma  the  tendon  reflexes  are  exaggerated,  the  muscles  spastic;  previous 
experience  justifies  the  diagnosis  of  the  excessively  dangerous  breaking  through 
of  the  blood  into  the  lateral  ventricle,  under  these  circumstances. 

Sometimes  a  short,  so-called  "stage  of  reaction"  pi'ecedes  coming  out  of 
the  coma;  during  this  the  apoplectic  becomes  restless,  sweats  profusely,  is 
somewhat  delirious,  complains  of  headache,  etc.  When  these  general  symp- 
toms have  passed  away  and  the  patient  is  again  conscious,  there  follows,  by 
degrees,  the  change  of  flaccid  into  spastic  hemiplegia  introduced  by  the  return 
of  the  tendon  reflexes  and  their  exaggeration  upon  the  paralyzed  side.  The 
Babinski  phenomenon  appears  o\\  this  last,  not  at  all  infrequently,  already  in 
the  coma;  in  traumatic  unilateral  cerebral  hemorrhages  I  have  been  able  many 
times  to  elicit  it,  even  in  the  hours  oust  after  the  accident.  With  the  develop- 
ment of  spasticity  there  is  often  a  rimiUiJjon  of  the  extent  of  the  paralysis. 
Indeed,  not  so  very  rarely,  the  disturbance  of  motility  may  disappear  in 
relatively  short  time.  This  is  the  case  when  the  hemorrhage  does  not  directly 
sever  the  cortico-spinal  tracts,  but  has  occurred  in  their  neighborhood,  for 
example,  in  the  lenticular  nucleus.  The  compression  to  which  the  neighbor- 
hood of  the  hemorrhagic  focus  is  exposed  for  some  time,  the  collateral  (edema, 
etc.,  are  to  be  held  responsible  in  such  cases  for  the  indirect  symptoms  of  inter- 
ference witli  the  motor  tracts.  Only  the  pareses  which  persist  6  to  8  months 
after  the  stroke  are  to  be  considered  as  direct  focal  symptoms,  and  prognosti- 
cally  to  be  estimated  accordingly. 

This  restitution  may  depend  upon  the  disappearance  of  the  so-called 
"Diaschisis."  By  this  expression — since  the  fundamental  work  of  v.  Monakort 
— we  denominate  as  sort  of  passive  shock:  the  absence  of  stimuli  which  are 
connected  with  the  destroyed  nerve  tracts  paralyze,  also,  anatomically  intact 
regions,  which  in  their  activity  have  adapted  themselves  to  these  stimuli.  The 
action  of  this  diaschisis  is  in  its  nature  fleeting,  temporary,  not   residual. 

Pathogenesis    and    Etiology   of   Cerebral    Hemorrhagic    Foci    and    Areas    of 

Softening 

Since  now  we  have  studied,  from  an  exclusively  symptomatologieal  point  of 
view  Hie  typical  clinical  pictures  of  capsular  hemiplegia  and  of  the  apoplectic 
attack  which  accompanies  it,  as  a  rule,  il  is  now  our  task  to  subject  to  a 
systematic  description  Hit'  different  pathological  conditions  which  lead  to  en- 
cephalorrhagia  and  encephalomalacia. 

1.  Arteriosclerotic  changes  hi  the  brain  vessels  take  the  first  place  in  the 
anatomical  substratum  of  cerebral  hemorrhage.  We  can  here  refer  to  the 
general  remarks  which  we  made  in  Lecture  W  (page  224),  in  speaking  "I 
cerebrospinal  arteriosclerosis.  We  would  emphasize  again  that  between  ar- 
teriosclerotic changes  of  the  peripheral  arteries  and  those  of  the  cerebral  ves 
gels  there  is  no  necessary  parallelism,  and  that  a  high  degree  of  alteration  of 
the  one  gel  iii  no  way  excludes  the  good  condition  of  the  other,  and  vice  versa. 


264  LECTURE    XVII 

Particular  interest,  however,  from  the  point  of  view  of  the  pathogenesis  of 
cerebral  hemorrhages  is  presented  by  the  characteristic  lesions  whose  exceed- 
ingly frequent  occurrence  in  the  small  and  medium-sized  cerebral  arteries  (par- 
ticularly, however,  in  the  lenticulo-striate  artery)  Charcot  and  Bouchard 
pointed  out  in  1868;  the  "miliary  aneurisms'"  which  usually  are  a  "sacciform" 
subvariety  of  vascular  dilatations,  have  at  most  a  caliber  of  1  mm  and  are 
often  found  in  very  great  numbers. 

Having  been  produced  by  the  influence  of  permanently  increased  blood 
pressure  (here  contracted  kidney  and  hypertrophy  of  the  heart,  frequent  ac- 
companiments of  arteriosclerosis  play  a  very  great  role),  they  are  exposed  to 
the  danger  of  bursting  upon  any  sudden  further  increase  of  the  blood  pressure. 
In  this  sense  act  all  influences  tending  to  produce  congestion,  which  either 
increase  the  arterial  blood  supply  to  the  brain  or  hinder  its  venous  return. 
On  this  account  the  following  physiological  conditions  furnish  the  most  fre- 
quent exciting  causes  of  cerebral  hemorrhages:  Digestion,  defecation,  coitus, 
psychical  excitement,  overexertion  of  any  sort.  As  to  traumatic  apoplexies, 
which  occur,  for  example,  after  a  fall  on  the  head,  it  is  to  be  remarked  that 
they  usually  affect  arteriosclerotic  individuals,  that  also  rupture  of  a  brain 
vessel  from  the  action  of  external  force,  as  a  rule,  presupposes  a  morbid  brittle- 
ness  of  the  cerebral  arteries.  The  apoplexies  depending  upon  arteriosclerosis 
usually  occur  beyond  the  fortieth  year.  Many  apoplectics,  long  before  the 
occurrence  of  cerebral  hemorrhage,  are  distinguished  by  the  so-called  "Hab- 
itus apoplecticus."  They  are  thick-set,  somewhat  corpulent  individuals,  with 
short  necks,  red  faces,  and  visibly  swollen  and  tortuous  temporal  arteries. 

The  pathologico-anatomical  picture  of  encephalorrhagia  is,  according  to 
the  stage  in  which  the  patient  comes  to  autopsy,  a  varied  one.  In  fresh 
hemorrhages  a  more  or  less  extended  (usually  walnut-size)  black-red  coagulum 
is  found.  About  this  the  brain  substance  is  broken  up  and  infiltrated  with 
blood.  In  somewhat  later  stages  the  saturation  of  the  neighborhood  with 
cedematous  fluid,  colored  citron-yellow  from  admixture  of  blood-coloring  mat- 
ter, is  characteristic.  Later,  the  following  changes  occur:  Contraction  of 
the  blood  clot,  liquefaction  and  absorption  of  the  destroyed  brain  substance, 
reactive  proliferation  of  the  glia  at  the  periphery  of  the  focus.  If  the  last 
has  become  small,  there  results  finally,  as  a  residuum  of  the  hemorrhage,  an 
ochre-colored  "apoplectic  scar."  If  it  is  a  large  extravasation,  an  "apo- 
plectic cyst''  filled  with  serous  fluid  is  left  behind.  The  most  important  sec- 
ondary lesion  of  capsular  hemorrhagic  foci  is  descending  degeneration  of  the 
pyramidal  tracts. 

Much  more  rarely  than  to  rupture  of  a  vessel,  arteriosclerosis  leads  to 
thrombotic  closure  of  one  of  the  brain  arteries.  In  such  cases  the  obliteration 
is  introduced  by  a  specially  intense  fibrocellular  proliferation  of  the  intima. 

2.  Si/philitic  Endarteritis  Obliterans. — In  this  vascular  disease,  studied  by 
Heubncr,  Fricdl/indcr.  and  others,  there  is  inflammatory  infiltration  of  the 
intima  with  round  cells,  as  well  as  considerable  increase  of  the  endothelium. 
By  the  intrusion  of  newly  formed  vessels  from  the  vasa  vasorum,  there  is  pro- 
duced within  the  diseased  brain  artery  an  organized  granulation  tissue,  which 
(occasionally  with  the  addition  of  thrombotic  blood  coagulation)   finally  com- 


EXCEPHALORRHAGIA    AND    EXCEPHALOMALACIA         265 

pletely  occludes  the  vessel  lumen  and  interrupts  the  blood  stream.  Now,  if 
no  collateral  circulation  occurs  (and  in  the  "end  arteries"  of  the  brain  this 
last  is  excluded),  the  brain  area,  cut  off  from  its  nourishment,  undergoes 
ischemic  necrosis  and  softening.  Fresh  areas  of  softening  are  white  or,  in 
consequence  of  infiltration  by  blood  from  the  vessels  of  the.  healthy  neighbor- 
ing tissue,  red  in  color.  Older  foci  take  on  a  yellow  or  brown  color,  on 
account  of  alterations  in  the  blood  pigment.  Syphilitic  endarteritis  affects 
on  the  average  younger  individuals  than  arteriosclerosis.  It  occurs  (upon 
a  hereditary  basis)  even  in  children;  most  of  the  cases,  however,  are  in  the 
third  and  fourth  decades  of  life.  Sometimes  it  leads  to  rupture  of  the  dis- 
eased vessel  instead  of  to  obliteration.  All  hemiplegias  in  young  individuals 
are  directly  suggestive  of  syphilis. 

Once  in  a  way  other  infectious  diseases  also  lead  to  affections  of  the  intima 
of  the  brain  arteries,  in  which  there  is  sometimes  formation  of  a  thrombus  in 
the  diseased  vessels;  the  very  rare  encephalomalacias  in  typhoid  fever,  diph- 
theria, etc.,  are  thus  explained. 

3.  Cerebral  Embolism. — This  variety  of  vascular  occlusion  depends  upon 
the  lodgment  of  a  coagulum  originating  somewhere  else  in  the  organism  in 
an  artery  of  the  brain.  The  place  of  origin  of  the  emboli  brought  to  the 
brain  is  usually  the  left  heart;  the  diseases  responsible  for  their  production 
are  vegetating  or  ulcerous  endocarditis  of  the  aortic  and  mitral  valves,  further 
heart  aneurisms  and  certain  cases  of  heart  weakness  in  which  thrombi  are  formed 
in  the  recess  or  in  the  auricles  of  the  left  heart,  and  later  portions  broken  off 
and  thrown  into  the  circulation.  Also  arteriosclerotic  disease  and  aneurisms 
of  the  aorta  and  of  the  carotids  occasionally  furnish  the  material  for  brain 
emboli.*  These  last,  in  by  far  the  majority  of  cases,  are  caught  in  the  Syl- 
vian artery,  occlude  it,  and  effect  destructive  softening  of  the  basal  ganglia 
and  the  internal  capsule — while  in  a  remarkable  manner  the  cortical  regions 
supplied  by  this  artery  often  escape  destruction  through  collateral  circulation. 
The  lift  Sylvian  artery  is  more  frequently  affected  than  the  right. f  It  is 
usually  young  individuals  in  whom  brain  emboli  occur.  As  exciting  factors 
those  producing  elevation  of  blood  pressure,  like  in  encephalorrhagia,  play 
an  unmistakable  role:  defecation,  coughing,  vomiting,  overexertion,  psychical 
(  xcitement,  coitus,  etc. 

Differential   Diagnosis   Between   Hemorrhage  mid  Softening 

It  is  sometimes  an  exceedingly  difficult  task  to  form  the  decision  as  to 
whether  an  apoplectic  attack  or  a  cerebral  hemiplegia  depends  upon  a  hemor- 
rhage or  is  the  consequence  of  an  embolic,  thrombotic,  or  endarteritic  vascular 
occlusion.  As  guiding  principles  in  this  differentia]  diagnosis,  flu-  following 
points  may  be  of  use  : 

*  As  to  septic  emboli  from  il»-  pulmonary  vein  (in  gangrene  of  Hit-  lung,  etc.),  see 
Brain     Misccss,   i.ecturc   MX. 

t  As  to  embolism  and  thrombosis  of  the  basilar  artery  ("acute  apoplectic  bulbar, 
paralysis"),  see  Lecture  V  1 1. 


266  LECTURE    XVII 

1.  Embolism  and  syphilitic  endarteritis  usually  affect  younger,  arterio- 
sclerotic thromboses  as  well  as  cerebral  hemorrhages,  older  individuals. 

2.  Heart  murmurs  speak  for  embolism;  it  is  to  be  remarked,  however,  that 
quite  frequently  with  the  appearance  of  the  apoplectic  attack,  heart  murmurs 
previously  present,  disappear,  and  do  not  reappear  for  some  time.  Hyper- 
trophy ox  the  left  ventricle  turns  the  balance  in  favor  of  hemorrhage. 

3.  The  urinary  findings  characteristic  for  chronic  interstitial  nephritis 
(increased  quantity,  small  amount  of  albumin,  hyaline,  and  granular  casts,  a 
very  few  leucocytes)  speak  rather  for  hemorrhagic  apoplexy.  Contracted 
kidney  occurs  in  about  30  per  cent,  of  the  cases  of  cerebral  hemorrhage,  while 
blood  casts  (an  expression  of  a  hemorrhagic  kidney  infarct)  point  to  embolism. 

4.  In  a  small  number  of  cases,  examination  of  the  eye  grounds  can  furnish 
diagnostic  aid,  namely,  when  hemorrhages  into  the  retina,  albuminuric  retinitis, 
or  embolism  of  the  central  artery  of  the  retina,  can  be  found.  The  last 
speaks  for  an  analogous  process  in  the  brain,  the  two  first  anomalies  for 
cerebral  hemorrhage. 

5.  The  forerunners  of  the  apoplectic  attack  (described  on  page  262)  are 
of  much  longer  duration  in  thrombotic  or  endarteritic  vascular  occlusions 
than  in  hemorrhage.  In  embolism  they  are  but  inconsiderable,  or  are  absent 
entirely. 

6.  In  the  attack  itself,  congestion  of  the  face  makes  probable  a  cerebral 
hemorrhage;  its  pallor,  a  closure  of  a  vessel;  nevertheless,  these  rules  admit 
of  exceptions  which  are  in  no  way  rare.  The  fall  of  the  temperature  at  the 
start  is  nearly  always  absent  in  vascular  occlusion.  Clonic  spasms  speak 
decidedly  in  favor  of  embolism,  particularly  when  they  are  unilateral.  Con- 
jugate deviation  with  relaxed  limbs  is  characteristic  of  hemorrhage.  Coma 
sometimes  is  absent  in  autochthonous  vascular  occlusion,  and  when  present 
is,  as  a  rule,  of  shorter  duration,  but  deeper  than  in  embolism;  very  pro- 
found and  long-continued  coma,  however,  indicates  bursting  of  an  artery. 
The  seizure  clears  up  most  slowly  in  this  last  lesion. 

7.  Accompaniment  of  a  right-sided  hemiplegia  appearing  after  the  ictus 
by  aphasia  (see  Lecture  XVIII)  speaks  in  general  for  cerebral  softening 
rather  than  cerebral  hemorrhage.  A  complete,  or  almost  complete,  disap- 
pearance of  the  hemiplegia  or  an  only  partial  development  of  this  (for  ex- 
ample, brachial  monoplegia)    speaks  in  the  same  sense. 

Apoplexies  which  leave  behind  a  hemiplegia  lasting  only  one  or  several 
days  should  always  arouse  a  suspicion  of  general  paresis.  In  young  individuals 
there  come  also  into  question,  as  already  said  (see  Lecture  VIII,  page  1-iO), 
the  apoplectiform  attacks  of  multiple  sclerosis. 

Prognosis 

The  clinical  distinction  between  the  several  anatomical  substrata  of  apo- 
plexies and  hemiplegias  is  not  only  of  scientific  interest,  but  also  of  great 
importance  prognostically.  Now,  the  decision  that  an  endarteritic  obstruction 
has  arisen  upon  a  syphilitic  basis,  arouses  the  hope  that  by  commencing  an 
energetic  anti-syphilitic  treatment  a  recurrence  will  be  prevented,  while  arterio- 


ENCEPHALORRHAGIA    AND    ENCEPHALOMALACIA        267 

sclerotic  thrombosis  gives  in  this  respect  a  very  dubious  outlook;  repeated 
attacks  are  here  the  rule;  in  many  cases  there  are,  finally,  bilateral  hemiplegias, 
pseudo-bulbar  paralytic  phenomena,  deep  dementia,  marasmus,  etc.  After 
passing  through  a  cerebral  hemorrhage  the  patient,  while  not  safe  from  recur- 
rences, is  much  less  threatened  by  them.  Also,  in  contradistinction  to  arterio- 
sclerotics with  brain  softening,  his  intelligence  remains  intact  after  the  seiz- 
ures. After  embolism  there  are  no  further  attacks,  as  a  rule.  Decisive  for 
the  prognosis  as  to  life  is  here  the  underlying  disease.  Further,  the  paralytic 
symptoms  resulting  from  cerebral  hemorrhages,  according  to  experience,  show 
a  much  greater  tendency  to  disappear  little  by  little  than  those  from  foci 
of  softening;  this  is  connected  with  the  fact  that  there  the  indirect  and  distant 
symptoms  play  an  important  role,  here  a  very  minor  one.  The  defect  symp- 
toms which  persist  about  a  month  after  the  occurrence  of  embolism  or  throm- 
bosis can  usually  be  considered  as  final,  while  in  hemorrhage  a  permanent  con- 
dition can  only  be  assumed  after  6  or  8  months.  If  there  is  decided  regression 
of  the  paralysis  immediately  after  recovering  from  a  sanguineous  apoplexy, 
this  is  prognostically  very  satisfactory.  The  appearance  of  contracture,  on 
the  other  hand,  excludes  finally  the  hope  of  complete  restoration. 

A  very  important  prognostic  question  is  as  to  whether  the  patient  will 
survive  the  apoplectic  attack  or  not,  in  case  he  does  not  die  at  once  ("apo- 
plexie  foudroyante").  That  a  spastic  condition  of  the  muscles  from  the  start 
clouds  the  prognosis,  we  have  already  said;  also,  we  have  emphasized  the  un- 
favorable indication  of  acute  decubitus.  Further,  very  bad  symptoms  are, 
long  duration  of  the  coma  (over  24  hours')  ;  its  increasing  depth  (ingravescent 
ftpoplexy);  the  occurrence  of  a  pneumonia;  finally,  "CheyTie-Stokes  respira- 
tion" (a  periodical  increase,  then  decrease,  of  the  depth  of  inspiration  which 
occasionally  can  lead  to  complete  intermissions  in  the  breathing). 

Treatment 

In  fresh  apoplexy,  after  we  have  taken  care  that  the  patient  is  put  to  bed, 
avoiding  all  jarring  (with  careful  supporting  of  the  head),  and  all  tighl 
clothing  has  been  loosened,  in  our  further  proceedings  the  differential  diag1- 
ttosis  between  hemorrhage  and  vascular  occlusion,  into  which  we  have  already 
entered  quite  thoroughly,  are  of  importance;  besides,  the  condition  of  the 
heart  and  the  vessels  must  always  be  taken  into  consideration.  The  time 
when  every  one  affected  by  an  apoplectic  stroke  was  bled  schematically  has 
gone  by.  We  consider  blood  letting  as  indicated  only  when  it  must  be  assumed 
that  there  is  cerebral  hemorrhage,  the  face  being  red,  the  pulse  tense.  In  many 
eases  the  application  of  leeches- behind  the  mastoid  processes  suffices;  when 
the  carotids  are  very  full  of  blood  and  there  is  great  congestion  of  the  face, 
however,  withdrawing  from  125  to  250  CC  of  blood  (venesection  or  puncture 
of  the  cubital  vein)  is  entirely  in  place.  On  the  other  hand,  if  the  clinical 
picture,  in  agreement  «ith  the  previous  history,  speaks  for  embolism  or  throm- 
bosis, if  the  face  is  pale,  the  pulse  is  weak  or  intermittent,  we  should  refrain 
from    bleeding,    and    must    make    Use    of    stimulants.       As    such,    there    come    into 

Consideration  subcutaneous  injections  of  ether,  acetic  ether,  camphorated  oil. 


268  LECTURE    XVII 

or  caffein,  as  well  as  oxygen  inhalations.  Tutting  an  ice-bag  upon  the  head 
of  the  apoplectic  is  a  measure  sanctioned  by  tradition  ;  the  possibility  that  in 
cerebral  hemorrhage  it  acts  through  the  skull  in  promoting  hemostasis  is, 
indeed,  very  problematic,  though  it  is  not  entirely  excluded;  in  embolism  and 
thrombosis,  however,  it  can  in  any  case  do  no  harm,  so  that  we  need  not  object 
to  this  ordinary  measure,  which  gives  a  certain  satisfaction  to  the  friends. 
Adrenalin  injections  are  to  be  avoided  in  cerebral  hemorrhage;  the  disadvan- 
tage of  the  considerable  increase  of  blood  pressure  which  they  cause  outweighs 
the  possible  advantages  of  a  vasoconstriction  (which,  if  we  draw  conclusions 
from  animal  experiments,  is  least  to  be  expected  in  the  brain  arteries).  That 
the  injection  of  ergot  preparations  is  able  to  stop  the  cerebral  hemorrhage 
is  very  questionable;  nevertheless,  there  is  no  objection  to  trying  them. 

From  the  start  we  should  exert  our  efforts  toward  the  avoidance  of  the 
dangers  of  acute  decubitus  and  pneumonia.  From  the  point  of  view  of  both 
of  these  it  is  to  be  recommended  when  the  coma  lasts  longer  than  2  or  3 
hours,  to  turn  the  patient  on  his  side  (naturally  very  carefully  and  with 
proper  assistance),  and  when  it  is  necessary,  after  a  few  more  hours,  to  change 
him  to  the  other  side.  Eventually  pneumatic  or  water  cushions.  The  skin 
exposed  to  bed-sores  is  bathed  from  time  to  time  with  alcohol.  Further,  it  is 
well,  with  a  wad  of  cotton  on  the  end  of  an  applicator,  to  mop  out  the  mucus 
which  tends  to  collect  in  the  pharynx,  from  time  to  time,  or  to  promote  its 
being  swallowed  by  encouraging  the  swallowing  reflex. 

The  retention  of  this  reflex  almost  without  exception,  as  a  rule,  permits 
giving  the  comatose  apoplectic  some  nourishment  without  having  to  use  the 
oesophageal  tube;  naturally  only  small  portions  must  be  given  at  one  time 
(beef-tea,  egg-nog,  "hvgiama,"  meat-juice,  olive  oil,  etc.,  a  tablespoonful  at 
a  time).  Also  analeptics  and  expectorants;  for  example,  strong  coffee  with 
aromatic  spirits  of  ammonia,  can  be  given  as  needed  from  time  to  time,  by 
the  mouth.  When  there  is  retention  of  urine,  we  should  not  forget  to  cathe- 
terize  at  proper  intervals;  in  the  more  frequent  incontinence,  the  utmost 
cleanliness  should  be  observed.  Enemata  are  needed  in  most  cases ;  for  "de- 
pletion by  the  intestine,"  instead  of  the  usual  injections  (soapsuds,  glycerin, 
etc.),  enemata  of  sulphate  of  sodium  or  sulphate  of  magnesium  solutions  can 
be  tried.  (After  emptying  the  bowel,  a  nutrient  enema,  consisting  of  gruel, 
yolk  of  egg  and  peptone  may  be  given.)  If  it  is  desired  to  act  depletingly 
not  only  through  the  intestine,  but  also  by  the  skin,  hot  packs  to  the  lower 
extremities  are  simplest;  more  energetic  effect  may  be  obtained,  however,  by 
the  application  of  mustard  plasters  to  the  calves. 

In  apoplexies  of  traumatic  origin,  with  persistent  or  ingravescent  coma, 
surgical  interference  (puncture,  or  even  incision  into  the  hemorrhagic  focus) 
has  been  practiced  occasionally  with  fortunate  outcome. 

In  the  stage  of  reaction  we  should  not  fear  to  combat  the  often  great 
jactitation  by  injections  of  morphine.  If  the  patient  regains  consciousness, 
it  is  our  first  task  to  explain  to  him  the  situation,  and  to  instill  into  him 
hope  and  equanimity  .by  pointing  out  the  improvement  that  is  to  be  expected. 
To  obtain  for  him  good  nights  he  should  be  given  the  alkaline  bromides, 
chloral,  codein,  veronal,  trional,  etc.     For  the  rest,  however,  with  the  excep- 


ENCEPHALORRHAGIA    AND    ENCEPHALOMALACIA        269 

tion  of  cases  of  syphilitic  etiology  (in  which  we  usually  give  iodipin  injections 
of  10  to  20  cc  of  a  25  per  cent,  solution  in  the  first  days  after  the  attack, 
and  begin  soon  after  with  injections  of  biniodide  of  mercury — see  Lecture  XIII, 
page  210),  it  is  better  to  avoid  further  medication.  Only  after  about  1-i 
days  the  arteriosclerotic  apoplectic  is  allowed  to  begin  a  course  of  iodide  of 
potassium,  as  we  have  described  it  in  Lecture  XV.  Naturally,  the  therapeutic 
and  special  dietetic  indications  given  for  arteriosclerosis  cerebro-spinalis  apply 
to  the  later  treatment  of  these  cases  in  the  fullest  degree.  During  the  earlier 
days  after  recovery  from  an  apoplectic  attack,  the  patient  is  kept  on  a  liquid 
and  semi-liquid  diet  (farinaceous  soups,  purees,  compotes,  pap,  etc.),  and  it  is 
sought  to  procure  regular  stools  without  straining,  by  mild  purgatives  (cas- 
eara,  purgen,  pil.  rhei,  comp.,  pulv.  glycyrrhiz.,  etc.). 

A  few  days  after  the  patient  has  regained  consciousness  very  careful  pas- 
sive movements  of  the  paralyzed  limbs,  with  gentle  massage  of  their  muscles, 
is  begun.  Above  everything,  it  is  necessary  to  oppose,  as  far  as  possible,  the 
formation  of  contractures  and  faulty  positions  of  the  extremities;  on  this 
account  we  should  see  to  it  that  the  foot  is  kept  continuously  in  a  position  at 
right  angles  to  the  leg,  and  that  the  arm  and  the  fingers  lie  as  far  as  possible 
stretched  out  (see  Lecture  III,  page  54).  Only  after  2  or  .'5  weeks  should 
electric  treatment  be  begun,  also  (and  apart  from  faradization  of  the  anesthetic 
skin  regions  with  the  wire  brush),  exclusively,  galvanism.  Stimulating  the 
muscles  by  the  faradic  current  is  contraindicated.  By  the  comparison  of 
otherwise  analogous  cases  of  cerebral  paralyses  which  have  been  treated  b}' 
faradism,  by  galvanism,  and  without  any  electro-therapy,  one  may  at  any 
time  convince  himself  that  the  faradization  of  the  muscles  can  favor  the 
development  of  hemiplegic  contractures.  The  constant  current  appears,  on  the 
other  hand,  when  it  is  used  particularly  to  stimulate  the  extensor  muscles  in 
the  upper,  the  peronei  and  flexors  of  the  knee  in  the  lower  extremity,  not 
only  to  encourage  voluntary  motor  innervation  (to  open  up  new  paths — 
"balmen"),  but  directly  to  oppose  the  occurrence  of  contractions. 

When  motion  has  returned  again  in  the  leg,  the  patient  can  be  gotten 
out  of  bed,  at  first  for  a  very  short  period.  For  a  number  of  weeks,  however, 
In-  should  spend  the  greater  part  of  the  day  ill  bed,  as  too  early  and  too 
frequent  attempts  at  walking  usually  favor  the  development  of  contractures. 
Only  very  gradually  should  long  remaining  up  and  going  about  be  permitted. 
The  arm  should,  at  the  start,  be  kept  in  a  sling  in  order  to  prevent  swelling 
and  cyanosis  of  the  dependent  hand  as  well  as  dragging  upon  the  joint,  which 
last  can  favor  the  development  of  arthropathies.  Now,  salt  baths  of  gradually 
{DCreasing  concentration  may  he  given,  provided  thai  they  are  neither  too 
fold  nor  too  hot  34  to  :i'r,  *'.  (93  to  !>.-.  F.).  During  the  bath,  cold 
Compresses  are  applied  to  the  patient's  head  to  avoid  congestion.  In  the  bath, 
systematic  exercises,  never  pushed  to  fatigue,  are  undertaken;  soon,  also, 
outside    the    bath.       In    these   less    stress    is    to    be    laid    upon    the   development    of 

itrength   than   upon  the  practicing  familiar  acts   (eating,  buttoning  and  mi 
buttoning  the  clothing,  writing,  etc.). 

Bui     rarely    orthopedic    after-treatment     of    hcmiplcgic    paralyses    and    eon 
fractures  comes  into  question.      At  most,  it   is  a  matter  of  supportive  apparatus 


270  LECTURE    XVII 

with   arrangements    for  extension ;   there    is   scarcely   ever   any   indication   for 
bloody  interventions,  as  tenotomy,  tendon  transplantation,  etc. 

(Supplementary.)  Atypical  and  Extracapsular  Hemiplegias 

Gentlemen:  It  is  necessary  now  to  call  your  attention  to  some  varieties 
of  cerebral  hemiplegia,  which  are  separated  more  or  less  widely,  pathologico- 
anatomically  and  semiologically  from  the  typical  capsular  hemiplegia  with 
whose  consideration  we  have  commenced  this  lecture.  If  in  this  task  I  do  not 
strictly  confine  myself  to  cerebral  hemorrhages  and  cerebral  softening,  never- 
theless this  bringing  in  of  other  pathologico-anatomical  conditions  is  amply 
justified  from  the  point  of  view  of  differential  diagnosis. 

1.  Cortical  Hemiplegia. — This  depends  chiefly  upon  embolic  or  thrombotic 
processes,  though  it  can  also  occur  from  tumors,  diseases  of  the  meninges  and 
the  bones  of  the  skull.  It  is,  on  account  of  the  great  extent  of  the  cortical 
motor  zone,  usually  incomplete,  that  is,  sparing  some  portions  of  the  limbs. 
When  it  begins  in  an  apoplectiform  manner  it  usually  causes  conjugate  devia- 
tion (see  page  262).  The  combination  with  cortical  aphasia  (see  Lecture 
XVIII)  is  very  frequent.  The  so-called  "intra-cortical"  hemiplegia  presents 
a  variety  of  cortical  hemiplegia.  Spielmeyer  has  shown,  namely,  that  chronic 
diseases  and  resulting  atrophies  of  the  cerebral  cortex  (for  instance,  in  epi- 
leptics) entirely  isolate  the  (for  the  rest  intact)  pyramidal  tracts  from  the 
other  cells  and  cell  associations  of  the  cortex,  and  can  hence  paralyze  them. 
Intra-cortical  hemiplegias  arise  naturally,  never  in  an  apoplectiform  manner, 
but  are  always  chronic  and  progressive. 

2.  Peduncular  Hemiplegia. — Hemorrhage  into  one  crus  cerebri  (into  the 
crusta  or  ventral  portion)  produces  (as  do  also  tumors  or  aneurisms  of  the 
same  region)  the  so-called  Weber's  symptom-complex,  or  alternating  oculo- 
motor hemiplegia.  In  this,  there  is  found  on  the  side  of  the  lesion,  a  paralysis 
of  the  oculo-motor;  on  the  opposite  side,  however,  a  paralysis  of  the  face  and 
the  extremities.  This  occurs  from  the  fact  that  the  root  fibers  of  the  third 
nerve  pass  close  to  the  pyramidal  bundles  which  are  proceeding  distally  in 
the  ventral  portion  of  the  crus,  but  do  not  cross  until  lower  down,  and  are 
destroyed  in  common  with  these  latter. 

3.  Pontine  Hemiplegia. — If  the  lesion  is  situated  a  little  farther  distally, 
namely  in  the  lower  third  of  the  pons,  there  results  from  this  another  alternat- 
ing hemiplegia,  the  so-called  "Millard-Gubler  symptom-complex"  (alternating 
facial  hemiplegia).  On  the  side  of  the  lesion  the  facial,  on  the  opposite  side 
the  limbs,  are  paralyzed.  The  explanation  is  furnished  by  the  following 
anatomical  relations :  the  pyramidal  fibers  intended  for  the  facial  undergo 
decussation  in  the  middle  third  of  the  pons,  while  the  rest  of  the  pyramidal 
fibers  only  cross  the  middle  line  distally  from  the  pons.  A  lesion  in  the  lower 
third  of  the  pons  varolii  hence  affects,  along  with  fibers  for  the  contralateral 
extremities,  those  intended   for  the   facial  muscles  of  the  same  side. 

4.  Hemiplegia  Cruciata,  an  excessive  rarity,  can  be  produced  by  hemor- 
rhages into  the  medulla  oblongata,  when  these  are  located  laterally  at  the 
pyramidal  crossing  through  which  the  tracts  for  the  arm  are  affected  before, 


EXCEPHALORRHAGIA    AXD    ENCEPHALOMALACIA        271 

those  for  the  leg  after,  passing  over  to  the  opposite  side.     The  paralysis  then 
affects  the  contralateral  arm  and  the  homolateral  leg. 

5.  "Lacunar  Hemiplegia"  has  already  been  mentioned  in  Lecture  XV,  when 
discussing  arteriosclerotic  brain  diseases.  This  form  of  hemiplegia  is  charac- 
terized, as  was  there  emphasized,  first  by  great  capacity  for  rapid  restitution 
and  slight  tendency  to  the  formation  of  contractures,  on  the  other  hand,  how- 
ever, by  a  manifest  tendency  to  new  attacks  in  the  originally  unaffected  hemi- 
sphere. In  the  last  instance  paretic  spastic  disturbances  occur  then,  also  in 
the  bilaterally  innervated  muscles  (see  above,  page  258),  which  manifest  them- 
selves in  pseudo-bulbar  phenomena.  Sensibility  and  skin  reflexes  are  scarcely 
disturbed  in  this  form ;  also,  when  it  begins  suddenly,  the  ictus  is  not  severe, 
consciousness  is  usually  preserved  or  but  slightly  clouded,  eventually  loss  of 
consciousness,  but  of  only  very  short  duration. 

6.  The  So-called  "Hemiplegia  Sine  Materia." — Cerebral  hemiplegias  have 
now  and  then  occurred  without  any  lesion  in  the  central  nervous  system  being 
discoverable  at  the  autopsy.  Many  times  it  has  been  in  nephritics  who  died 
in  uremia.  The  uremic  poison  seems  here  for  some  reason  entirely  obscure  to 
us,  to  have  acted  in  producing  one-sided  paralysis.  The  older  authors  thought 
of  one-sided  vascular  spasm  and  spoke  of  "serous  apoplexy."  Other  cases 
of  so-called  hemiplegia  sine  materia  exposed  in  the  older  literature,  however, 
are  probably  to  be  considered  as  unrecognized  lacunar  hemiplegias. 

7.  The  So-called  "Homolateral  Hemiplegia." — There  are  found  in  the 
literature  some  observations  in  which  the  disease  focus  has  been  found,  not  on 
the  opposite  side,  but  upon  the  same  side  as  the  hemiplegic  paralysis.  Usually 
there  have  of  course  been  mistakes  in  clinical  or  pathologico-anatomical  ob- 
servation: so,  namely  after  embolism,  as  already  said,  spasmodic  movements 
mi  v  occur  in  the  paralyzed  extremities  immediately  after  the  ictus;  these  may 
be  wrongly  interpreted  as  signs  of  voluntary  muscular  power,  while  the  con- 
tralateral extremities,  in  consequence  of  the  coma,  lie  motionless  and  arc 
considered  to  be  paralyzed.  Further,  on  autopsy  a  gross  lesion  of  one  hemi- 
iphere,  which,  however,  does  not  involve  the  motor  tracts,  may  so  fix  the  at- 
tention of  the  examiner  that  he  overlooks  inconspicuous  foci  located  in  the 
pons  or  medulla  of  the  other  side.  However,  after  eliminating  these  cases 
erroneously  designated  as  homolateral  hemiplegia,  there  still  remain  a  few 
unassailable  observations:  on  several  occasions  an  absence  of  the  pyramidal 
decussation,  a  relatively  rare  fiber  anomaly,  has  been  found  in  such  cases. 


LECTURE  XVIII 

Aphasia,  Apraxia  and  Agnosia 

Gentlemen  :  In  our  lecture  to-day  we  will  occupy  ourselves  with  the  ex- 
ceedingly instructive  and  interesting  disturbances,  which  can  appear  in  dif- 
ferent brain  diseases  in  consequence  of  the  more  or  less  elective  destruction  of 
a  definite  "memory."  Where  it  is  a  "memory"  necessary  for  the  speech  ar- 
rangement, we  see  an  aphasia  result  from  its  elimination;  if  there  are  dis- 
turbances of  associated  memories,  affecting  movements  adapted  to  an  end  but 
not  in  the  service  of  speech,  the  clinical  picture  of  apraxia  occurs;  if,  finally, 
the  recognition  of  objects  of  any  sort  is  interfered  with  or  rendered  impossible, 
we  speak  of  agnosia.  In  each  of  these  phenomena,  however,  we  have  to  con- 
struct a  large  number  of  symptomatological  sub-groups,  which,  for  the  deter- 
mination of  the  seat  and  the  nature  of  the  underlying  pathological  processes 
are  in  part  of  decisive  importance. 

A.     Aphasia 

Already,  in  1825,  the  French  clinician  Bouillaud  emphasized  the  difference 
which  existed  between  a  paralysis  of  the  speech  mechanisms  and  another  con- 
dition in  which  these  muscles  had  suffered  loss,  not  in  their  power  to  act  in 
itself,  but  only  in  their  applicability  to  the  service  of  forming  words.  Eleven 
years  later  Marc  Dax,  based  upon  the  frequent  coincidence  of  such  a  loss  of 
speech  with  right  hemiplegia,  placed  the  seat  of  "word  memory"  ("memoire 
verbale")  in  the  left  hemisphere.  The  anatomical  proof  of  the  general  correct- 
ness of  this  view  was  furnished  first  by  P.  Broca  ( 1801-1865),  in  that  he  showed 
that  the  motor  speech  center  was  to  be  sought  in  the  foot  of  the  third  lower 
frontal  convolution  on  the  left  side.  Further,  he  recognized  exceptional  cases 
with  right-sided  location  of  this  center.  Soon  after,  observations  on  a  different 
sort  of  aphasia,  in  which  the  lesions  occurred  in  the  left  temporal  lobe  and  which 
were  characterized  not  by  the  impossibility  of  forming  words,  but  by  substitu- 
tions, leaving  out  and  mutilation  of  words  ("paraphasia")  and  jargon,  were 
made  known.  The  English  neurologist  Charlton  Bastian,  laid  stress  on  the  fact 
that  these  patients  also  presented  disturbances  in  the  understanding  of  words,  in 
that  they  perceived  what  was  spoken  to  them  only  as  noise,  but  could  not 
understand  it  as  speech.  Finally,  in  1874,  Wernicke  proved  that  this  last 
phenomenon,  which  he  called  "sensory  aphasia,"  occurred  through  the  de- 
struction of  a  "center  for  the  memory  of  sounds"  in  the  superior  left  temporal 
convolution,  and  that  the  accompanying  paraphasic  phenomena  are  explain- 
able from  the  loss  of  the  regulating  influence  which  this  "Wernicke's  zone 
(as  we  call  it  to-day),  exercises  upon  Broca's  convolution. 

•272 


APHASIA,    APRAXIA    AND    AGNOSIA  273 

Newer  investigations  make  it  exceedingly  probable  that  Broca's  center  is 
not  1  mited  to  the  foot  of  the  third  frontal  convolution,  but  extends  to  the 
neighboring  parts  of  the  Island  of  Rcil,  the  second  frontal  convolution  and 
the  precentral  gyrus.  Further,  v.  Monakow  has  shown  that  Broca's  center 
cannot  be  considered  simply  as  a  depot  for  "kinesthetic"  memory  pictures* 
for  the  synergies  necessary  for  speech.  The  Zurich  neurologist  demands  with 
ju,sti(  .  a  greater  consideration  of  the  dynamic  factor  along  with  the  anatomi- 
cal one,  in  the  valuation  of  facts  in  the  aphasia  question.  In  this  he  stands 
upon  the  ground  of  the  so-called  "Diaschisis  theory."  This  last  assumes  that 
when  any  part  of  the  cortex  of  the  brain  is  eliminated,  not  only  this  itself  is 
inhibited  in  its  function,  but  also  a  sort  of  shock-like  inhibition  is  exerted  upon 
other  cortical  areas  connected  with  it.  This  shock  is,  however,  not  to  be  con- 
sidered as  active;  rather  does  the  absence  of  stimuli  from  the  destroyed  center 
simp]  affect  a  passive  paralysis  of  those  cortical  regions  which  have  regulated 
them  lv»8  in  their  activity  by  these  stimuli,  but  which,  through  the  new  con- 
ditions, have  suddenly  been  put  out  of  setting.  This  "Diaschisis"  action  is  in 
its  i  nee  of  temporary  nature.-  In  the  extended  Broca's  region  lies  now  a 
place  of  production  of  motor  aphasia,  in  that,  this  part  of  the  cortex  acts  as 
director  over  other  cortical  regions,  which  probably  distributed  through  large 
portions  of  both  hemispheres  affect  the  proper  carrying  out  of  the  acts  of 
speech.  Hence,  lesions  of  Broca's  zone  alone  (chiefly  traumatic)  produce 
motor  aphasia  only  temporarily  and  in  the  restitution  of  the  most  important 
speech  functions  in  such  cases,  the  passing  away  of  diaschisis  is  to  be  per- 
ceived— the  cortical  apparatus  coming  into  question  learns  in  time  to  work  even 
withou'  the  regulation  of  Broca's  center.  Before  v.  Monakow,  the  disap- 
pearan  ■  of  aphasic  phenomena  was  explained  in  general  by  the  vicarious  action 
of  symmetrica]  parts  of  the  other  hemisphere  (to  a  certain  extent,  "reserve 
fcenters"  iews  appear  to  me,  however,  to  indicate  an  important  progress. 

This  last  can  in  no  way  be  said  of  the  attempt  of  P.  Marie  to  deny  to  Broca's 
zone  any  significance  in  the  production  of  motor  aphasia. 

In  the  r  i  cases  of  Broca's  aphasia  in  which  the  autopsy  disclosed  no 
disease  of  tin  lot  of  the  third  frontal  convolution,  there  may  have  been  very 
fine  alterations,  for  example,  senile  cortical  atrophies,  only  to  be  discovered 
microscopicall  if  not  purely  functional  aphasias;  there  is,  for  example,  a 
hysterical  aphi  ia  studied  by  Marinesco  among  others.  As  to  the  still  rarer 
observations  in  which  clinically  there  was  no  aphasia  but  Broca's  cortical  area 
on  autopsy  is  found  destroyed,  we  by  no  means  need  to  overturn  the  whole 
mroca-Wernicke  localization  theory  on  this  account.  The  following  reflection 
suffices:  The  left-handed  person  has  his  speech  sphere  as  Broca  emphasized, 
on  the  right  side.  Nevertheless*  he  has  seen  left-handed  people  also  become 
botor-aphasic  when  the  left  lowest  frontal  convolution  was  destroyed.  Even 
so,  is  the  left-sided  cortical  localization  of  the  psychical  speech  mechanism  in 

light-handed   people  a  rule,  which  does   not    exclude  certain   exceptions,   and  on 

this  account  it  is  no  wonder  thai   right-handed  people  sometimes  have  not  be- 

*  Thai  is,  memory  pictures,  which  render  j>< i---.il i]»-  the  repetition  of  formerlj  carried  oui 
analogous  movements. 


274. 


LECTURE    XVIII 


come  aphasic  after  destruction  of  the  left  lowest  frontal  convolution: — they 
had  had,  probably,  their  speech  regulating  centers  exceptionally  developed  on 
the  right,  since  in  general  an  originally  bilateral  plan  for  this  cortical  sphere- 
can  be  assumed  with  the  greatest  probability.  In  destruction  of  Broca,s  con- 
volution there  may  have  been  aphasia,  which  later,  however,  disappeared.  , 
that  the  person  making  the  autopsy  if  he  has  no  accurate  information  as  to 
the  past  history,  registers  a  lesion  in  the  third  frontal  convolution  in  a  | 
not  aphasic. 

THE  PATHOLOGICAL  PHYSIOLOGY  OF  THE  APHASIC 
PHENOMENA 

I  beg  you  now  to  carefully  observe  Fig.  82.  You  will  note  in  it  the  fol- 
lowing: 

The  center  for  motor  aphasia  is  connected  not  only  with  the  cortical  centers 
for  the  lip,  tongue,  and  larynx  muscles ;  that   for  sensory  aphasia  not   only 


Fig.  S2. 

The  Cortical  Speech  Centers  and  Their  Connections. 

B  =  Brora's  Center.  W  =  Wernicke's  Center.  L  =  Reading  Center.  S  =  Writing  Center. 
a  =  Third  (lower)  Frontal  Convolution,  li  =  First  (upper)  Temporal  Convolution.  c  = 
Anterior  Central  Convolution,    d  =  Angular  Gyrus. 


with  the  auditory  cortical  zone,  but  these  two  cortical  regions  communicate 
by  association  fibers,  1,  with  one  another,  2,  with  the  higher  psychical  centers 
of  the  frontal  region  (the  so-called  "conceptual  center"),  3,  with  the  cortical 
apparatus  which  render  writing  and  reading  possible.     The  left  angular  gyrus 


APHASIA,    APRAXIA    AND    AGNOSIA 


27.5 


presents  a  reading  center  proper,  the  memory  field  for  the  recognition  of 
letters,  whose  destruction  produces  alexia,  word  blindness.  The  writing  center, 
on  the  other  hand,  contrary  to  the  former  view,  coincides  with  the  "hand  and 
finger"  center  of  the  anterior  central  gyrus. 

The  network  of  association  fibers  indicated  schematically  in  our  figure 
comes  into  consideration  for  those  psychical  functions  which  we  can  designate 
shortly  as  "internal  speech."  Under  this  designation  we  understand  everything 
which  must  go  on  beneath  the  threshold  of  consciousness  in  our  brains,  before 
we  translate  a  thought  into  words  and  project  these  words  outward  by  way 
of  mouth  or  writing,  or  before  we  can  take  other  cognizance  of  spoken  or 
written  expressions.     This  internal  speech  develops  ontogenetically,  in  that  the 


BROCA'S 
CENTER. 


SPEECH  MUSCLES 
CENTER. 


Seat  of  a  Cortical  Motor  Aphasia  (  \,  Blocks  1.  -.  ■).  and  1)  and  of  a  Sub-Cortical  Motor 
Aphasia  (B,  Blocks  only  t). 

child  first  repeats  words  heard,  then  connects  a  concept  ion  with  them,  then 
upon  the  emergence  of  tin's  conception  reproduces  tin-  words  himself,  later, 
on  learning  to  write  and  read, 'connects  definite  symbols  with  the  individual 
sounds,  etc. 

Of  decisive  importance  for  the  understanding  of  the  aphasia  question,  is 
now  the  fact,  that  all  the  association  fibers  in  tin  service  of  internal  speech 
run  through  the  brain  cortex,  while  those  neurones  serving  external  speech 
make  their  way  through  the  medullary  substance.  In  Fig.  88  you  see  this 
schematically  indicated,  and  learn  from  it  that  a  cortical  lesion  of  Broca's 
tenter  (A)    musi    produci    more  symptoms  than  a  subcortical  one  (B).     By 


276  LECTURE    XVIII 

the  first,  the  association  net-work  for  internal  speech  is  broken,  by  the  last, 
however,  in  no  way  touched,  so  that  subcortical  motor  aphasia  indicates  a 
pure  aphasia  an  exclusive  suppression  of  external  speech,  a  simple  "word  dumb- 
ness." Mutatis  mutandis  in  the  same  manner  cortical  sensory  aphasia  differ- 
entiates itself  from  the  subcortical  pure  "word  deafness." 

When  now,  we  turn  to  the  study  of  the  individual  aphasic  symptom-com- 
plexes we  will  proceed  according  to  the  following  pkui : 

1.  The  four  chief  forms  of  Aphasia. 

(a)  Cortical  motor  aphasia,  Broca's  aphasia. 

(b)  Subcortical  motor  aphasia,  pure  word  dumbness. 

(c)  Cortical  sensory  aphasia,  Wernicke's  aphasia. 

(d)  Subcortical  sensory  aphasia,  pure  word  deafness. 

2.  Other  forms  of  Aphasia. 

(a)  Total  aphasia. 

(b)  Conduction  aphasia. 

(c)  Transcortical  aphasias. 

(d)  Aphasias  of  "single  senses." 

3.  Other  symptom-complexes  resembling  the  aphasic  phenomena. 

(a)  Alexia. 

(b)  Agraphia. 

(c)  Amusia. 

(d)  Amimia. 

CORTICAL   MOTOR   APHASIA,   BROCA'S    APHASIA 

Through  the  destruction  of  the  motor  cortical  zone  of  Broca,  it  becomes 
impossible  for  the  patient  to  translate  conceptions  into  words.  Indeed,  such 
a  patient  can  emit  sounds  and  his  speech  muscles  are  not  paralyzed  like  those 
of  one  with  anarthria  as  a  result  of  bulbar  paralysis ;  still  their  regulated  co- 
operation necessary  for  speech  has  become  impossible.  The  French  language 
can  express  the  difference  between  this  expressive  speech  disturbance  of  the 
motor  aphasic  and  the  articulators  speech  disturbance  of  the  anarthric  in 
much  shorter  and  more  complete  fashion  than  the  German :  "1'aphasique  ne 
sait  plus  parler,  1'anarthrique  ne  peut  plus  parler"  (the  aphasic  does  not 
know  how  to  speak  any  longer,  the  anarthric  cannot  speak  any  longer).  There 
are  graduated  differences,  hence,  a  quite  considerable  variety  of  clinical  pic- 
tures occurs.  Either  the  patient  has  entirely  lost  the  power  of  speech,  has  re- 
tained a  few  stereotyped  expressions,  or,  in  slight  cases,  there  has  remained  to 
him  a  rudimentary  ability  of  emitting  speech,  so  that,  for  example,  he  uses  all 
verbs  in  the  infinitive  ("negro  fashion")  or  even  leaves  them  out  ("dispatch 
style"). 

Along  with  the  loss  or  the  disturbance  of  speech,  writing  is  also  disturbed 
to  a  corresponding  degree.  Abi'ity  to  copy  can,  however,  be  retained,  as  a 
tracing  of  the  letters  which  is  accomplished  without  the  assistance  of  memory 
pictures.     Reading,  on  the  other  hand,  is  always  more  or  less  impaired  and  often 


APHASIA,    APRAXIA    AND    AGNOSIA  277 

special  methods  are  needed  to  make  evident  this  disturbance.  So,  for  example, 
printed  words  «in  be  understood,  written  ones,  on  the  other  hand,  cannot ;  or 
the  understanding  of  words  may  be  suspended  if  (with  the  help  of  the  well- 
known  child's  block  alphabet)  the  syllables  are  separated  and  arranged  at 
horizontal  or  vertical  intervals.  The  understanding  of  speech  appears  in  a 
general  manner  undisturbed ;  when,  however,  one  speaks  very  quickly  to  the 
patient,  or  uses  a  complicated  sentence  in  the  conversation,  ofie  can  make  out 
an  increased  difficulty  of  understanding.  Though,  however,  the  disturbances 
of  speaking  and  writing  stand  out  most  prominently,  nevertheless  the  de- 
struction of  a  nodal  point  in  the  cortical  association  net  has  also  not  left 
reading  and  understanding  entirely  unmolested.  To  have  indicated  this  last 
point  was  namely,  the  service  of  my  teacher,  Dejerine. 

SUBCORTICAL  MOTOR-APHASIA,  PURE  WORD  DUMBNESS 

Here,  as  is  visible  in  Fig.  82,  the  path  between  Broca's  center  and  that  for 
the  speech  muscles  has  been  destroyed.  Internal  speech,  reading,  writing, 
understanding  of  speech,  are  intact.  Though  the  patient  cannot  bring  out  the 
words  in  contradistinction  to  patients  with  Broca's  aphasia,  he  has  preserved 
the  corresponding  motor  memory  pictures;  he  can  even  with  his  fingers  give 
tin-  number  of  syllables  in  the  expression  which  he  endeavors  in  vain  to  emit. 
( Dcjcrinc-IJchthrim  phenomenon.) 

Pure  subcortical  motor  aphasia  has  now  certain  relationships  with  those 
conditions  with  which  we  have  become  acquainted  as  anarthria,  or  dysarthria 
(Lectures  VII  and  XV).  Common  to  both  symptom-complexes  is  the  reten- 
tion of  interna]  speech;  while,  however,  in  anarthria  the  formation  of  sounds 
is  disturbed,  we  find  in  pure  motor  aphasia  that  the  formation  of  the  word 
from  the  individual  sounds  is  impaired.  Of  these  sounds,  often  a  large  number 
can  be  emitted  explosively  as  the  so-called  "word  debris."  For  the  inex- 
perienced, confusion  may  easily  arise  between  these  two  conditions. 

CORTICAL    SENSORY    APHASIA,    WERNICKE'S    APHASIA 

As  already  said,  the  sound  pictures  rendering  possible  the  understanding 
of  speech,  are  preserved  in  the  posterior  sensory  or  Wernicke's  speech  center 
of  the  left  superior  temporal  convolution.  Destruction  of  this  center  causes, 
hence,  no  loss  of  the  power  of  speaking,  but  of  the  understanding  of  speech, 
no  expressive,  but  a  perceptive  disturbance.  In  this,  the  patient,  indeed,  hears 
the  words  spoken  to  him,  but  cannot  comprehend  their  sense,  since  they  no 
longer  rouse  into  consciousness  the  conceptions  corresponding  to  them.  His 
native  language  sounds  to  him  as  a  foreign  language,  of  which  he  knows  noth- 
ing, or  lias  learned  but  little,  does  to  the  healthy  person.  That  sensory  aphasia 
is  often  accompanied  bv  paraphasia,  that  such  patients  continually  make  mis- 
takes in  speaking  and  instead  of  the  words  which  are  proper,  emit  other,  often 
similarly  sounding  words,  is  connected  with  the  following:  We  unconsciously 
first  pronounce  internally  the  word  which  we  wish  to  speak  by  means  of  Wer- 
nicke's sound  picture  center.     When,  now,  this  no  longer  functionates,  there 


278  LECTURE    XVIII 

is  easily  the  emission  of  wrong  words,  which  the  patient  again  cannot  notice, 
since  in  consequence  of  his  sensory  aphasia,  lie  does  not  understand  his  own 
words. 

Where  the  understanding  of  speech  is  not  entirely  lost  but  considerably 
affected,  it  is  particularly  the  familiar  expressions  (such  as:  "How  are  you?" 
"What  is  your  name?"  etc.)  whose  sense  the  patient  is  still  able  to  comprehend. 
Sometimes  the  patient  also  guesses  the  sense  of  the  sentence  from  one  char- 
acteristic word  which  he  understands.  If  now,  we  vary  the  sense  of  the  sen- 
tence while  retaining  this  characteristic  word,  a  corresponding  modification 
of  the  answers  of  the  patient  does  not  occur.  For  example,  "Have  you  chil- 
dren?" "Yes."  "How  many  children?"  "Yes."  "Where  are  your  children?" 
"Yes." 

In  contradistinction  to  the  motor  aphasic  who  speaks  little  or  not  at  all, 
the  patient  with  Wernicke's  aphasia  usually  talks  a  great  deal,  but  badly.  We 
denominate  his  paraphasia  verbal  when  he  confuses  words  (for  instance, 
says  "dog"  instead  of  "bed")  ;  as  literal  when  he  confuses  letters  (for  example, 
"winnow"  instead  of  "window"),  while  we  speak  of  "Jargon  aphasia"  when  he 
brings  out  a  succession  of  senseless  syllables.  In  this  last  case  there  is  some- 
times a  clinging  to  one  syllable  which  is  repeated  over  and  over — "persever- 
ation." 

The  understanding  of  writing  is  also  suspended  in  cortical  sensory  aphasia 
or  at  least  impaired.  In  complete  "word  blindness"  the  patient  can  no  longer 
read,  and  the  letters  are  for  him  only  senseless  black  figures  upon  a  white  ground, 
which,  however,  he  may  trace  without  understanding.  Writing  voluntarily  or 
after  dictation  is,  on  the  other  hand,  lost  or  impaired  (this  last  in  the  form  of 
literal  or  verbal  paragraphia).  Other  symbols  besides  letters  such  patients 
may  still  understand,  for  example,  very  often  numbers,  and  further,  they  can 
play  cards  and  dominoes.  A  patient  of  Dejerine's  who  could  not  read  the 
letters  R.  F.,  said  instantly  "Republique  Francaise,"  as  soon  as  the  coat  of 
arms  was  drawn  about  the  two  letters. 

SUBCORTICAL    SENSORY    APHASIA— PURE   WORD   DEAFNESS 

In  this  very  rare  form,  of  which  I  have  so  far  been  able  to  observe  no  case, 
and  a  knowledge  of  which  we  owe,  among  others,  to  Dejerine,  Wernicke,  Liep- 
mann  and  Sachs,  internal  speech  is  intact,  reading  and  writing  may  be  possible 
without  disturbance  and  only  the  understanding  of  speech  is  suspended.  Be- 
sides this,  paraphasia  is  absent  in  pure  word  deafness  since  the  sensory  corti7 
cal  center  is  still  able  to  exert  its  stimulating  and  controlling  action  upon  the 
motor  center.  This  form  occurs  only  through  the  cutting  off  of  the  other- 
wise intact  Wernicke's  zone  from  the  stimuli  coming  from  the  general  audi- 
tory center. 

I  might  here  introduce  an  anatomical  point  not  unimportant  for  localiza- 
tion. It  has  indeed  been  proved  by  autopsy  that  a  subcortical  lesion  of  the 
left  temporal  lobe  can  be  the  substratum  of  pure  word  deafness.  Still,  in- 
complete destruction  of  the  sensory  speech  center  can  probably  also  produce 
pure  word  deafness,  just  as  occasionally  incomplete  disturbances  of  the  motor 


APHASIA,    APRAXIA    AND    AGNOSIA  279 

speech  center  can  cause  pure  word  dumbness.  I  assume  that  in  such  cases 
the  association  fibers  between  the  cortical  field  of  the  cochlear  nerves  and 
Wernicke's  center,  or  respectively  between  Broca's  convolution  and  the  speech 
muscle  center,  are  interrupted  intracortically,  without  on  this  account  the 
function  of  the  speech  centers  under  consideration  having  been  destroyed. 

TOTAL    APHASIA 

We  speak  of  total  aphasia  when  a  pathological  process  has  destroyed  the 
anterior  as  well  as  the  posterior  speech  center  and  on  this  account  ability  to 
speak  as  well  understanding  of  speech,  and  also  ability  to  read  and  to  write 
are  destroyed. 

CONDUCTION    APHASIA 

This  designation  Wernicke  has  introduced  for  certain  speech  disturbances 
which  are  characterized  by  paraphasia,  paragraphia,  and  impairment  of  speech 
repetition.  He  assumes  that  here  there  is  interruption  of  the  direct  communi- 
cation between  the  sensory  and  the  motor  speech  centers,  in  which  particularly 
a  lesion  in  the  Island  of  Keil  would  come  into  consideration.  This  clinico- 
anatomical  hypothesis,  however,  has  been  proven  untenable  by  v.  Monakow. 

TRANSCORTICAL   APHASIAS 

According  to  Lichtheim  these  arise  by  the  blocking  of  the  paths  between 
the  conceptional  center  on  the  one  hand  and  Broca's  or  Wernicke's  center  on 
the  other.  Hence,  a  motor  and  a  sensory  variety  are  differentiated.  In  the 
first  only  voluntary  speech  and  writing  are  suspended  or  impaired;  speech 
repetition,  reading  aloud,  writing  from  dictation  are,  on  the  other  hand,  re- 
tained. In  the  latter  there  is  more  or  less  pronounced  word  deafness  and 
alexia  along  with  paraphasia  and  paragraphia,  but  speech  repetition,  reading 
aloud  and  writing  from  dictation,  may  be  possible,  although  without  under- 
standing. With  Dejerine,  I  consider  these  forms  only  as  intermediate  stages  of 
cortical  aphasias  which  are  improving.  As  an  ''attenuated  form  of  transcor- 
tical motor  aphasia"  Liepmann  has  designated  the  so-called  amnestic  aphasia 
or  verbal  amnesia,  in  which  finding  the  word  has  become  very  difficult,  the 
suggested  word,  however,  is  at  once  recognized  as  correct  and  is  repeated 
Bmoothly  and  perfectly.  Further,  chiefly  substantives  and  verbs  for  bhe  con- 
crete are  not  found,  while  the  forms  of  speech  and  further  inflection  and 
declension  are  preserved. 

APHASIAS    OF    SINGLE    SENSES 

Best  known  is  the  optic  aphasia  of  Freud;  for  objects  simply  held  before 
the  patient   he  cannot  find  a  name,  which,  however,  promptly  occurs  to  him 

when    he    is    given    an    opportunity    to    observe    the    object    through    sonic    other 

tense  than  that  of  sight,  for  example,  to  feel  a  spoon,  to  hear  a  bell,  to  taste 
a  piece  of  sugar.     At  the  basis  of  this  disturbance  lies  an  interruption  of  the 


280  LECTURE    XVIII 

connection  between  the  centers  for  the  recognition  of  objects  in  the  occipital 
lobe  and  Wernicke's  sound  memory  center  in  the  temporal  lobe.  The  existence 
of  an  optic  aphasia  in  the  strict  sense  of  the  word  is,  however,  according  to 
Gustav  Wolff,  not  definitely  proved — neither  are  the  other  analogous  single 
sense  aphasias  (auditory,  tactile  aphasia).  As  a  matter  of  fact,  many  of 
these  observations  have  entirely  lacked  certain  proof  that  the  patients  act- 
ually had  recognized  the  seen,  touched,  heard  things,  and  only  could  not  find 
their  names,  also  that  they  were  free  from  mind  blindness,  mind  deafness  and 
touch  agnosia.      (See  below.) 

ALEXIA 

This  disturbance  leads  in  slight  cases  to  blindness  for  letters,  in  severe 
ones  to  word  blindness,  and  can,  apart  from  accompanying  cortical  aphasias, 
appear  more  or  less  independently.  If  a  focus  is  seated  in  the  reading  center 
of  the  left  angular  gyrus,  the  alexia  is  accompanied  by  agraphia,  since  the 
association  fibers  to  the  center  for  writing  movements  in  the  precentral  con- 
volution are  interrupted.  We  speak  then  of  "cortical  alexia."  If,  on  the 
other  hand,  the  focus  is  subcortical,  beneath  the  angular  gyrus,  so  that  only 
to  the  optic  word  pictures,  which  come  from  the  visual  cortex  in  the  cuneus, 
the  entrance  is  closed,  there  occurs  "pure  alexia."  Also,  conditions  which 
mutatis  mutandis  correspond  to  those  which  are  present  in  cortical  and  sub- 
cortical aphasias. 

AGRAPHIA 

Disturbance  of  the  writing  center,  shown  in  Fig.  82,  causes  no  isolated 
agraphia,  but  a  paralysis  of  the  right  hand.  With  the  left,  however,  such  a 
patient  may  be  able  to  write  or  to  learn  quickly  to  do  so,  provided  that  his 
Broca's,  Wernicke's  and  reading  centers  have  remained  intact.  Isolated 
agraphia  we  will  indeed  become  acquainted  with  later  as  a  component  of 
apraxia. 

AMUSIA 

As  motor  amusia  the  inability  to  sing,  as  sensory,  the  inability  to  appre- 
ciate a  melody,  is  designated.  Also  musical  alexia  or  blindness  for  notes  is 
usually  herein  included.  The  amusic  disturbances  are  rare  accompaniments 
of  the  corresponding  forms  of  aphasia.  The  musical  centers  appear  to  be 
situated  very  close  to  the  corresponding  speech  centers,  but  do  not  entirely 
coincide  with  them. 

AMIMIA 

The  condition  that  in  the  patient  also  expression  and  gesture  speech  is 
lost  (motor  amimia)  and  even  understanding  for  this  has  disappeared  is  found 
in  total  aphasia.  If  in  this  manner  all  possibility  for  comprehending  one's 
fellowrs  is  excluded,  we  speak  of  asemia  or  asymbolia. 


APHASIA,    APKAXIA    AND    AGNOSIA 


281 


EXAMINATION    FOR    APHASIA    AND    DIAGNOSIS    OF    THE 
INDIVIDUAL    FORMS 

When  we  have  before  us  an  aphasic  patient  we  must  bend  our  endeavors 
toward  making  as  rapid  a  diagnosis  as  possible  of  the  form  of  aphasia  pres- 
ent. After  that,  we  will  take  up  its  symptomatological  study.  I  would  recom- 
mend that  you  proceed  according  to  the  following  scheme  which  I  have  pre- 
pared after  the  manner  of  the  botanical  determinative  books.* 


Understanding  of   < 
Speech. 


Retained  :  Mot.  Aphasia  - 


Lost:  Sens.  Aphasia 


Writing  retained  : 
Subcortical 


Writing  lost: 


Understanding  of 
writing  retained  : 
Subcortical 


Understanding  of 

writing  lost : 


Ability  to  repeat  words 
retained :  Transcorti- 
cal 

Ability  to  repeat  words 
lost:  Cortical 


Ability  to  repeat  words 
retained  :  Transcorti- 
cal 

Ability  to  repeat  words 
lost:  Cortical 


In  general  it  is,  especially  for  the  beginner,  of  advantage  in  the  thorough  ex- 
amination of  an  aphasic  to  stick  to  one  of  the  schemata  proposed  by  different 
investigators  and  to  carefully  register  the  result  of  the  examination.  I  give 
you  here  for  this  purpose  the  Stewart  examination  schema  somewhat  modified 
by  myself,  which  you  will  find  useful  in  all  cases: 

1.  Can  the  patient  spontaneously  give  utterance  to  understandable  words? 
How  extensive  is  his  vocabulary?     Can  he  utter  all  words,  or  only  some? 

The  patient  is  allowed  to  talk  spontaneously  and  it  is  observed  whether  lie 
beaks  fluently,  whether  he  misplaces  words  or  syllables,  whether  he  speaks 
in  connected  sentences,  or  talks  unintelligible  stuff. 

2.  Does  he  understand  Hie  words  which  he  hears?  He  is  asked  to  touch 
alternately  his  nose,  his  car,  his  eye,  his  chin,  etc.,  in  order  to  test  his  under- 
standing of  substantives.  He  is  then  requested  to  whistle,  to  smile,  to  close 
his  eves,  etc.,  by  which  his  understanding  for  verbs  is  determined.  It  should 
in  observed  if  he  carries  out  the  first  order  correctly,  then,  however,  continu- 
ally repeats  the  same  act.  even  when  requested  to  do  something  else.  If  there 
is  no  trouble  in  carrying  out  these  simple  orders,  he  is  given  more  compli 
cated  sentences,  for  example,  "I  beg  you,  when  I  have  come  around  to  the' 
other  side  of  the  bed,  to  touch  my  arm  twice  with  your  left  hand."1 

.'5.    Does    he    understand    written    questions    or    orders    which    are    placed    lie 

in  this  I  ignore  the  problematical  "conduction  aphasia,"  and  on  account  of  it--  small  prac- 
tical importance  I  have  put  in  parentheses  what  is  said  about  tin-  differentia]  diagnosis  of  the 
M-called  "transcortical"  aphasia. 


282  LECTURE    XVIII 

fore  him?  In  testing  this,  simple  sentences  are  first  used  (for  example,  "How- 
old  are  you?"  "Show  me  your  tongue."  "Give  me  your  left  hand")  and 
only  after  this,  more  complicated  constructions.  Further,  not  only  hand- 
writing, but  also  print,  is  used  and  it  is  noticed  whether  separation  of  the 
individual  syllables  or  their  unusual  arrangement  disturbs  this  understanding 
of  them   (see  page  277). 

4.  Can  he  write  spontaneously? 

If  his  right  hand  is  paralyzed,  he  may  try  with  his  left  hand.  It  is  observed 
whether  he  scribbles  senseless  signs. 

5.  Can  he  transcribe  printed  letters  into  written  ones,  and  vice  versa? 
For  this  test  one  of  the  so-called  "letter  games"  with  which  short  words 

like  "Monday,"  or  short  sentences  as  "Where  is  the  tree,"  are  put  together, 
or  allowed  to  be  put  together,  is  used. 

6.  Can  he  write  from  dictation? 

7.  Can  he  find  objects  whose  names  he  hears? 

A  heap  of  objects,  like  a  key,  a  piece  of  money,  a  lead  pencil,  a  match,  etc., 
are  placed  before  him  and  he  is  solicited  to  pick  up  one  at  a  time. 

8.  Can  he  repeat  words  which  he  hears? 

He  is  first  tried  with  simple  words  and  sentences,  for  example,  "Cat," 
"Dog,"  "Sister,"  "Good  morning,"  etc.  If  he  has  lost  the  power  to  repeat 
words  spoken  before  him,  it  is  ascertained  whether  the  patient  can  sing,  what 
is  sung  for  him,  which  is  sometimes  the  case. 

9.  Can  he  name  objects  which  he  sees? 

Different  objects  are  pointed  to  (see  under  7)  and  he  is  asked  "What  is 
that?" 

10.  Can  he  read  aloud? 

11.  Can  the  patient,  in  case  it  is  impossible  for  him  to  pronounce  a  word, 
give  the  number  of  its  syllables  on  his  fingers? 

12.  Does  he  understand  gestures  and  mimic  movements? 

Without  speaking  to  him,  he  is  solicited  to  imitate  touching  the  nose 
spreading  the  fingers,  sticking  out  the  tongue,  etc. 

In  order  not  to  fatigue  the  patient  these  different  tests  are  to  be  under- 
taken not  all  at  one  sitting,  but  with  extensive  periods  of  rest  between.  In 
polyglot  patients  it  is  well  to  test  the  different  languages  separately  when 
possible.      The  same  thing  applies  to  dialect  and  to  written  speech. 

PROGNOSIS   OF   APHASIA 

The  estimation  of  the  etiological  factors  is  very  important  in  the  first 
place  (whether  hemorrhage,  embolism,  tumor,  trauma,  etc.).  Of  decisive  im- 
portance is  the  intellectual  condition.  The  onset  of  dementia  makes  the  hope 
of  disappearance  of  the  aphasic  disturbances  illusory.  Particular  emphasis, 
however,  is  to  be  attached  to  the  prognostic  differences  as  to  restoration  of 
function  which  appear  to  exist  according  to  the  form  of  the  aphasia  or  accord- 
ing to  the  localization  of  its  anatomieal  substratum.  The  best  outlook  is  pre- 
sented by  pure  wrord  dumbness,  while  the  severe  cortical  aphasias  with  com- 
plete loss  of  spoken  mid  written  speech  present  little  prospect  of  improvement. 


APHASIA,    APRAXIA    AND    AGNOSIA  2«:i 

In  sensory  aphasias  the  prognosis  is  the  worse  the  more  intensely  marked 
paraphasia  and  jargon  aphasia  are.  Alexia  and  agraphia  in  improvement  of 
Wernicke's  aphasia,  show  themselves  much  more  obstinate  than  word  deafness. 

Treat nn  nt 

The  exercise  treatment  of  these  conditions  furnishes  the  greatest  test  of 
patience  which  comes  to  the  physician.  Gutzmann  properly  warns  impera- 
tively against  beginning  the  exercises  too  early.  We  should  wait  at  least  half 
a  year  after  the  cessation  of  the  stormy  symptoms  and  the  entire  disappearance 
of  all  other  disturbances.  If  exercise  is  commenced  too  early  in  apoplectics, 
there  is  the  danger  that  during  the  exercises,  in  consequence  of  the  exertion, 
a  new  hemorrhage  may  occur.  Gutzmann  usually  first  prescribes  the  exer- 
cises when,  after  one  or  two  years,  there  is  no  further  spontaneous  improve- 
ment of  speech.  If  they  are  applied  earlier,  it  is  very  difficult  to  decide  how 
much  is  owing  to  the  exercise  treatment.  Gutzmann  further  lays  the  great- 
est stress  upon  slow  progress,  as  only  by  this  can  the  patient  be  kept  in  good 
spirits,  which  is  absolutely  necessary  for  the  result  of  the  exercise  treatment. 
Sensory  aphasics  with  speech  impulsion  must  first  practice  holding  their 
tongues,  endeavoring  to  regain  again  to  some  extent  the  normal  inhibition  by 
the  will.  Even  in  the  severe,  prognostically  unfavorable,  Broca's  aphasias, 
treatment  should  not  be  given  up,  since  it  is  of  great  importance  for  the  pa- 
tient to  give  expression  to  his  will,  at  least  by  a  few  words,  even  when  they  are 
used  ungrammatically.  By  this,  the  lability  of  mood  which  not  rarely  be- 
comes exaggerated  to  outbreaks  of  rage,  is  combated.  There  occurs,  from 
the  fact  that  the  aphasic  is  not  in  a  condition  to  give  expression  to  his 
thoughts,  wishes  and  conceptions,  an  internal  tension,  for  which  even  a  rudi- 
mentary ability  to  speak,  gives  an  outlet.  Also  to  this  same  end.  such  pa- 
tients must  systematically  practice  gesture  speech.  A  picture  book  with  sim- 
ple representations  of  all  the  conceptions  and  wishes  lying  within  the  circle 
of  thought  »f  the  patient,  should  always  accompany  him,  so  that  he  can  make 
known  his  wishes  to  those  surrounding  him  by  indicative  gestures,  though  when 
apraxia  is  present,  the  descriptive  gestures  cannot  be  learned. 

B.     Apraxia 

As  apraxia*  we  denominate,  since  the  monumental  work  of  the  Berlin 
psychiatrist  Ijcjiniaini  which  first  appeared  in  1900,  a  disturbance  in  which 
the  extremities,  especially  the  hands,  are  capable  of  executing  correct  single 
movements,  but  not  those  movements  directed  to  a  definite  end.  A  patienl 
effected  with  this  disturbance  has  lost  the  memory  of  the  synergies  and  com- 
hiii.il inns  of  single  movements  necessary  for  the  proper  use,  for  example,  of  a 
pair  of  scissors,  I'm'  beckoning,  for  shaking  hands,  etc.  lie  sticks  a  tooth- 
brush like  a  cigar  into  his  mouth,  and  so  on.  There  are  hence  great  analogies 
with  motor  aphasia,  or  to  better  express  it.  aphasia  from  a  physiological  point 

Other  names,  .'is  Asymbolia,  Parektropia,  Parekinesia,  .ire  applied  only  by  ;i  tew  authors, 
ami  have  nut  come  into  general  use. 


284  LECTURE    XVIII 

of  view  can  be  considered  a  special  form  of  apraxia,  as  apraxia  of  the  speech 
apparatus. 

We  distinguish  three  varieties  of  apractic  disturbances : 

1.     IDEATORY    APRAXIA 

The  idea-plan  for  the  composite  act  to  be  carried  out  is  disturbed,  so  that 
the  resulting  unsuitable  action  gives  the  impression  of  extreme  mental  dis- 
traction. Liepmann  cities  the  following  typical  examples:  The  patient  sticks 
a  match  along  with  his  cigar  into  his  mouth  instead  of  striking  it ;  in  sealing 
a  letter,  puts  the  seal  in  the  flame  and  then  presses  it  on  the  stick  of  sealing 
wax;  attempts  to  cut  off  the  end  of  his  cigar  by  wedging  it  between  the  match 
case  and  its  cover.  The  single  acts  which  make  up  these  perverted  maneuvers 
are  in  general  correctly  performed.  This  ideatory  apraxia  usually  appears 
only  in  complicated  movements  and  in  proportion  to  their  complexity.  The 
limbs  are,  as  a  rule,  equally  affected ;  it  is  not  that  they  respond  incorrectly 
to  the  will,  but  they  receive  perverted  impulses  from  it. 

2.     IDEO-MOTOR   APRAXIA 

In  this  form,  both  the  plan  of  movement  and  also  the  limb  center  with  its 
content  of  kinesthetic  memory  pictures  are  intact,  but  their  connections  are 
severed.  Simple  acts  for  whose  accomplishment  memories  preserved  in  the 
limb  centers  suffice,  are  correctly  executed ;  only  those  requiring  a  great  num- 
ber of  successive  movements  are  incorrectly  accomplished,  since  the  proper  di- 
rections and  commands  are  no  longer  carried  from  the  ideation  centers  to  the 
limb  center  which,  to  a  certain  extent,  has  become  autonomous  with  regard  to 
the  whole  brain.  Liepmann  calls  these  forms  of  apraxia  the  "ideo-kinetic," 
Heilbronner,  the  "transcortical."  They  can  affect  single  limbs  or  one  half  of 
the  body.  According  to  Liepmann  the  following  kinds  of  faulty  reactions 
occur: 

a.  Movements  which  do  not  resemble  any  purposive  movements,  flouri>h- 
ing  the  hand,  spreading  the  fingers,  so-called  amorphous  movements. 

b.  Executing  the  wrong  movements.*  (Beckoning  instead  of  threatening, 
taking  hold  of  the  ear  instead  of  the  nose,  etc.) 

c.  The  movement  occurs  in  another  set  of  muscles  ;  standing  stiff  instead 
of  giving  the  hand,  which  may  simulate  leaving  out  of  movements. 

</.   Often  motor  helplessness  and  actual  abandonment  of  movement  appears. 
In  many  cases  of  the  faulty  reactions  there  is  decided  perseveration  (com- 
pare the  analogous  condition  in  aphasia,  page  278). 


3. 

MOTOR  APRAXIA 

In 

this,  the 

mot 

jr  innerv 

it  ion  is  disturbed  by  an 

injury 

to  the  limb  center 

itself 

which   is 

not 

sufficient 

to   paralyze   this,   but 

enough 

to   extinguish 

its 

*  Can  also  be  called 

"Parapraxia." 

APHASIA,    APRAXIA    AND    AGNOSIA  285 

kinesthetic  memory  pictures.  On  this  account  the  movements  of  the  extremity 
are  carried  out  as  if  the  patient  was  trying  them  for  the  first  time,  so  the 
coarse  movements  are  very  awkward,  the  finer  acts,  however  (writing,  thread- 
ing a  needle,  sewing),  cannot  be  executed.  Liepmann  calls  this  variety  of 
apraxia  the  '"limb  kinetic,"  Heilbrunner,  the  "cortical,"  Kleist  speaks  of 
"innervation    apraxia." 

The  Diagnosis  of  Apraxia 

As  a  scheme  for  examination  I  recommend  to  you  that  of  Dromard  and 
Pascal.  The  patient  is  solicited  first  by  simple  spoken  orders,  eventually  by 
making  before  him  the  necessary  gestures,  to  carry  out  the  following  cate- 
gories of  movements: 

1.  Auto-kinetic  movements:  Walking,  standing  up,  sitting  down,  dressing 
and  undressing  himself. 

2.  Simple  movements:  Close  the  eyes,  open  the  mouth,  show  the  tongue, 
cross  the  arms,  raise  the  right  hand,  stretch  out  the  left  hand,  spread  the 
fingers,  raise  the  right  leg,  etc. 

3.  Movements  requiring  reflection:  Show  the  right  eye,  touch  the  left  ear, 
twist  the  beard,  scratch  the  head,  etc. 

4.  Expressive  movements:  Throw  a  kiss,  "thumb  the  nose,"  make  the  mili- 
tary salute,  cross  himself,  etc. 

5.  Descriptive  movements :  Show  how  one  plays  the  piano,  turn  an  organ, 
grind  coffee,  how  to  catch  a  fly,  etc. 

6.  Complicated  movements:  Present  a  glass  of  water,  tie  a  knot,  light  a 
candle,  seal  an  envelope. 

In  order  to  diagnose  apraxia  it  is  necessary  to  exclude  word  deafness,  that 
is,  to  determine  that  the  patient  understands  the  command;  also  agnosia,  that 
is,  to  make  suit  that  he  correctly  recognizes  the  objects,  for  instance,  docs 
not  take  tin-  toothbrush  for  a  cigar.  Against  confusion  with  atactic,  choreic 
and  athetoid  movements  one  must  naturally  he  on  his  guard. 

In  the  diagnosis  of  the  lesions  upon  which  the  apractic  syniptoni-coni- 
nlexcs  depend,  the  following  rules  are  applicable: 

a.  Ideatory  apraxia  occurs  in  diffuse  processes  (senile  dementia,  pro- 
gressive paralysis,  diffuse  arteriosclerosis  of  the  brain). 

b.  Motor  ("limh  kinetic")  apraxia  depends,  as  already  said,  upon  dis- 
ease processes  in  the  cortical  centers  of  the  extremity  in  question,  which  with- 
out being  sufficienl  in  paralyze  them,  nevertheless,  destroy  their  kinesthetic 
pernor y  pictures. 

c.  Ideo  motor  (ideo-kinetic)  apraxia  occurs  under  two  different  conditions: 

1.  First  in  genera]  when  the  sensori-motor  cortex  is  retained  iii  its  in- 
tegrity, indeed,  hut  is  cut  oil'  from  its  connection  with  other  parts  of  the 
cortex,  namely  from  the  areas  for  word  and  objeel  conception  in  the  temporal 
and  occipital  lolies,  hence,  foci  in  the  parietal  lobe  cause  all  apraxia  of  the 
opposite  hand. 

2.  An  ideo-motor  apraxia  of  the  left  hand  is  also  observed  in  destruction 
of  the  lihers  of  Hie  corpus  callosum   in  which   its  sensori-motor  cortical   field 


286  LECTURE    XVIII 

in  the  right  hemisphere  remains  intact,  but  it  is  deprived  of  its  relations  with 
the  sensori-motor  cortical  field  of  the  left  hemisphere.  The  left  hemisphere 
controls  also   (at  least   in   right-handed  people)   the  activity  of  the  right. 

SPECIAL    FORMS    OF    APRAXIA 

Along  with  the  gross  apractic  symptom-complexes  there  occur  also  special 
apractic  disturbances,  of  which  the  most  important  is  apractic  agraphia, 
which,  in  contradistinction  to  the  aphasic  form,  can  occur  as  an  isolated 
phenomenon.  I  would  mention  further  the  observations  preserved  in  the 
literature  of  apraxia  of  lid  closing,  apraxia  of  the  head  muscles,  apractic 
disturbances  in  the  finger  speech  of  deaf  mutes,  and  "instrumental  amusia" 
in  musicians ;  also  amimia,  already  mentioned,  may  be  thought  of  again  in 
this  connection. 

C.    Agnosia 

If,  in  spite  of  undisturbed  perception  by  .the  senses,  the  recognition  of 
objects  of  the  external  world  is  impossible,  we  speak  of  agnosia.  If  we 
would  extend  this  conception  to  the  conventional  phonetic  or  graphic  speech 
symbols,  we  would  have  to  denominate  the  phenomena  of  word  deafness  and 
alexia,  already  known  to  us  as  special  instances  of  auditory  and  optic  agnosia. 
This,  however,  contradicts  the  usual  use  of  the  word  which,  under  "agnosia," 
understands  only  "object  agnosia."  We  distinguish  auditory,  optic  and  tac- 
tile agnosia ;  there  are  also  taste  and  smell  agnosias,  but  they  are  so  difficult 
to  differentiate  from  ageusic  and  anosmic  disturbances,  and  in  any  case  so 
rare,  that  they  are  practically  of  no  importance. 

1.    AUDITORY    AGNOSIA:    MIND    DEAFNESS 

In  this  (in  consequence  of  disturbed  associations  between  the  areas  for 
auditory  sense  perceptions  and  the  "concept  center")  the  identification  of 
auditory  impressions  is  suspended.  With  closed  eyes  such  a  patient  is  unable 
to  recognize  a  watch  by  its  ticking,  a  dog  by  his  bark,  a  bunch  of  keys  by 
their  rattling,  a  stream  of  water  by  its  splashing,  etc.  Mind  deafness  is  an 
accompaniment  of  sensory  aphasia  in  large  foci  in  the  left  temporal  lobe. 

2.     OPTIC     AGNOSIA:     MIND     BLINDNESS 

From  the  cortical  end  stations  of  the  optic  tract  (in  the  so-called  cuneus 
on  the  median  surface  of  the  occipital  lobe),  association  fibers  run  to  the 
lateral  convex  surface  of  the  occipital  lobe.  If  the  cuneus  is  the  perception 
center  for  the  optic  "impressions,  these  parts  of  the  cortex  represent  the  optic 
conception  centers.  In  them  the  memory  pictures  of  the  meaning  of  an  object 
seen  are  laid  up.  Bilateral  destruction  of  these  cortical  optic-memory  fields 
(or  of  the  association  fibers  passing  to  them  from  the  cuneus)  produces  "mind 
blindness,"  in  which  the  patients  see  the  objects  as  flat  or  physical  pictures. 


APHASIA,    APRAXIA    AND    AGNOSIA  287 

but  cannot  recognize  them.  Where  unilateral  disease  of  the  occipital  lobe 
has  produced  optic  agnosia  it  has  been  in  the  form  of  tumors  which  also 
must  have  injured  the  opposite  occipital  lobe  through  pressure.  If  we  show 
a  mind-blind  person  an  object  (spoon,  pencil,  bottle,  key,  hat,  box,  scissors, 
photograph,  glove,  etc.),  he  can  tell  its  form;  often  also,  its  color;  he  can 
further  pick  out  similar  objects  without  difficulty ;  he  has,  however,  lost  the 
understanding  of  the  use  of  the  object. 

3.    TACTILE    AGNOSIA 

This  form  of  agnosia  occurs  in  disease  foci  of  the  posterior  central  convo- 
lution and  the  parts  of  the  parietal  lobe  just  behind  it,  when  the  patient  is  re- 
quired to  recognize  objects  by  touching  them  with  the  opposite  hand.  The 
testing  must  particularly  be  carried  out  with  each  hand.  Naturally,  not  only 
must  the  eyes  of  the  person  being  investigated  be  blindfolded,  but  also  character- 
istic noises  (for  example,  the  rattling  of  a  bunch  of  keys)  must  be  avoided. 
In  diagnosing  tactile  agnosia  it  is  further  necessary  to  determine  that  the 
individual  modalities  of  superficial  and  deep  sensibility  (movement,  touch, 
pressure,  cold,  warm  sensations,  etc.)  are  not  disturbed  to  such  an  extent 
as  by  this  alone  to  render  recognition  illusory. 


LECTURE   XIX 

Tumor  Formations,  Inflammations,  and  Disturbances 
of  Circulation  in  the  Brain  and  its  Membranes 

A.   Brain  Tumor 

Gentlemen  :  When  we  speak  of  brain  tumor  in  the  clinical  sense,  we 
understand  thereby  not  only  neoplasms  arising  from  the  brain  substance  itself, 
but  all  intracranial  tumor  formations  which  act  upon  the  brain;  that  is, 
also  those  which  take  their  origin  from  the  meninges  and  from  the  bones  of 
the  skull,  and  to  these  tumors  we  reckon  along  with  the  neoplasms  proper, 
in  a  pathologico-anatomical  sense;  also  tuberculous  and  syphilitic  granulomata, 
as  well  as  many  parasites  (as  cysticercus  and  echinococcus),  and,  finally,  other 
space-reducing  processes  within  the  skull ;  for  example,  aneurisms  of  the 
Sylvian  artery  or  of  the  carotid,  certain  hematomata  of  the  dura  mater,  also 
the  so-called  "serous  meningitis,"  cannot  be  separated  clinically  from  brain 
tumor. 

In  serous  meningitis  there  is  a  great  serous  effusion  into  the  subarachnoid 
space  leading  to  symptoms  of  pressure  upon  the  contents  of  the  skull ;  that  is, 
the  analogue  of  serous  pleurisy  or  peritonitis.  It  is  often  accompanied  with 
increase  of  the  ventricular  fluid  (acquired  hydrocephalus),  and  develops  partly 
in  consequence  of  infectious  diseases  (for  example,  measles  or  pneumonia), 
partly  upon  a  syphilitic  basis,  partly  in  connection  with  inflammatory  dis- 
eases of  the  ear  or  nose,  partly  after  injuries  to  the  skull;  often,  however,  its 
etiology  cannot  be  made  out.  Along  with  the  diffuse  forms  of  serous  menin- 
gitis there  occur  circumscribed  encapsulated  collections  of  fluid  in  the  sub- 
arachnoid space,  usually  at  some  place  upon  the  convexity  of  the  brain ;  such 
observations  have,  for  example,  been  reported  by  Fcdor  Krause,  Iselin,  and 
myself.  Only  exceptionally  does  serous  meningitis  run  a  course  similar  to  that 
of  purulent  meningitis  (see  below),  although  much  milder;  usually,  however, 
it  gives  the  symptomatology  of  brain  tumor,  and  hence  furnishes  the  chief 
contingent  of  the  so-called  "pseudo-tumor  cerebri."  (Under  the  last  classi- 
fication would  fall  also  a  number  of  cases  which  furnish  clinically  the  typical 
picture  of  a  brain  tumor,  but  on  autopsy  show  neither  a  tumor  nor  meningeal 
effusion,  and  which  have  been  conceived  to  be  the  result  of  a  molecular  "brain 
swelling." — Reichardt  and  others.) 

Of  tumors  in  the  interior  of  the  skull,  glioma,  gliosarcoma,  and  sarcoma 
of  the  brain  occur  most  frequently;  sarcomata  often  take  their  origin  also 
from  the  meninges  or  from  the  connective  tissue  envelope  of  the  nerve  roots 
(particularly  the  facial  and  the  auditory,  so-called  "tumors  of  the  cerebello- 

288 


BRAIN    TUMOR  289 

pontine  angle").  Fibromata  have  also  been  observed  here.  From  the  inner 
surface  of  the  dura  arises  endothelioma,  which  sometimes  spreads  out  as  a 
flat  mass  ('"endotheliome  en  nappe"),  sometimes,  however,  forms  a  spherical 
tumor;  where  this  last,  through  the  inclusion  of  calcareous  material,  presents 
a  sandy  cross  section  and  very  firm  consistence,  we  speak  of  psammoma. 
Upon  the  clivus  at  the  base  of  the  skull  through  proliferation  of  cells  of  the 
chorda  dorsalis,  which  arc  there  included  in  the  bone  substance,  chordoma 
arises,  while  osteoma  and  chondroma  from  other  places  in  the  skull  bones  can 
intrude  into  the  cranial  cavity.  By  epidermal  germ  dispersion  in  the  interior 
of  the  skull,  and  indeed  in  the  ventricles,  arise  cholesteatoma,  dermoid  cyst 
and  teratoma.  Very  rare  is  lipoma,  which  can  develop  in  the  region  of  the 
corpus  callosum ;  still  rarer,  epithelioma  of  the  ventricular  ependyma,  of  which 
I  have  observed  one  case.  Relatively  more  frequent  is  adenoma  of  the  hypo- 
physis. The  other  epithelial  tumors  of  the  interior  of  the  skull  have  scarcely 
ever  arisen  autochthonously — rather  have  been  introduced  by  metastasis ;  this 
applies  especially  to  the  carcinomata.  Among  the  infectious  granulation 
tumors,  solitary  tubercle  of  the  brain  takes  the  first  place,  particularly  in 
childhood.  Not  very  rare  is  also  the  gumma,  which  most  frequently  arises 
from  the  membranes  of  the  cerebral  convexity.  As  a  rarity,  actinomycosis 
has  been  described.  Finally,  as  to  the  parasitic  cysts,  thanks  to  the  strict 
meat  inspection,  they  rarely  occur  in  our  neighborhood  any  more:  the  cystic 
stage  of  the  taenia  solium  (cj'sticercus)  is,  on  the  contrary,  occasionally  ob- 
served in  the  brain  in  the  north  of  Europe,  in  the  south  the  echinococcus 
("hydatid  cyst")  somewhat  more  frequently.  As  far  as  they  are  not  parasitic 
or  infectious  new  growths,  the  etiology  of  brain  tumors  is  naturally  just  as 
obscure  to  us  as  that  of  neoplasms  in  general;  nevertheless,  it  is  certain  that 
among  exciting  causes  skull  trauma  has  strikingly  often  been  found  in  the 
history.      Men  are  more  frequently  affected  than  women. 

Symptovuitology 

The  morbid  manifestations  produced  by  brain  tumors  may  be  divided 
into  general  symptoms  and  focal  symptoms.  The  first  are  to  he  interpreted 
predominantly  as  "brain  pressure"  phenomena;  that  is,  produced  by  the 
narrowing  of  the  space  within  tin-  cranial  cavity  and  the  resulting  compression 
of  the  brain,  and  hence,  to  a  certain  extent,  independent  of  the  seal  of  the 
tumor.  The  last,  on  the  contrary,  are  directly  connected  with  the  special 
location  of  the  disease  proofs,  and  hence  put  us  in  a  position  to  diagnose 
the  seat  of  trouble,  which  is  naturally  of  prime  importance  in  the  eventuality 
of  guxgical  interference. 

Among  the  different  brain  pressure  phenomena,  headache  occupies  the 
first  place;  this  is  usually  characterized  by  terrible  severity  as  soon  as  the 
space  within  the  skull  has  been  narrowed  to  a  considerable  degree.  It  can  be 
continuous,  hut  is  usually  described  as  remittent  exacerbating.  A  migraine- 
Eke  headache,  occurring  paroxysmally  and  accompanied  with  vomiting,  also 
occurs,  especially  in  the  early  stages.  Its  character  is  usually  grinding 
and   boring,   and   it    is   usually    felt    all   oxer    the   head.      In    those   cases,   how- 


290  LECTURE    XIX 

ever,  in  which  it  is  localized  in  a  definite  region,  this  by  no  means  necessarily 
corresponds  to  the  seat  of  the  tumor;  so,  indeed,  in  tumors  of  the  posterior 
fossa  of  the  skull  there  is  often  pain  limited  to  the  back  of  the  neck  and  head; 
sometimes,  however,  on  the  contrary,  frontal  headache.  Sensitiveness  on  per- 
cussion or  to  pressure,  localized  at  some  point  on  the  skull,  has  great  value, 
as  it  not  rarely  corresponds  exactly  with  the  disease  focus  below.  One  should, 
however,  never  be  satisfied  with  the  result  of  one  examination,  but  should  only 
use  diagnostieally  such  symptoms  as  have  proved  to  be  constant  on  long 
observation.  A  in  no  way  frequent,  but  very  important,  symptom  on  skull 
percussion  is  further  the  finding  over  a  definite  region  of  the  skull  a  "cracked 
pot"  sound  which  seems  to  depend  upon  a  lack  of  firmness  in  the  skull  sutures, 
and  is  most  frequent  in  young  patients. 

Upon  Roentgenological  examination,  a  definite  bulging  and  thinning  of 
the  bone  in  the  part  of  the  skull,  corresponding  to  a  tumor,  is  sometimes  to 
be  made  out;  on  the  other  hand,  there  is  sometimes  thickening  of  the  skull 
walls,  especially  in  the  region  of  such  tumors  as  arise  from  the  dura.  Only  a 
very  small  percentage  of  brain  tumors  manifest  themselves  in  the  X-ray 
picture,  by  destruction  of  the  skull  wall;  most  frequently  hypophyseal  tumors 
which  erode  the  sella  turcica.  A  particularly  interesting  brain  pressure  symp- 
tom is  dilatation  of  the  veins  of  the  diploe  (SchiiUer).  Scarcely  recognizable 
in  the  normal  X-ray  picture,  in  brain  tumors,  they  are  so  distended  that  they 
appear  as  wide  stripes  on  the  plate.  This  dilatation  can  be  plainest  in  the 
neighborhood    of   tumors. 

"Cerebral  vomiting"  is  a  very  frequent  brain  pressure  symptom.  It  ap- 
pears either  very  suddenly  without  any  warning,  explosively,  or  a  short  period 
of  nausea,  or  an  attack  of  vertigo,  precedes  it ;  in  any  case,  however,  it  is 
independent  of  taking  food.  For  the  rest,  vertiginous  phenomena  which  are 
to  be  considered  as  general  brain  pressure  symptoms  are  much  less  frequent 
in  intracranial  tumors  than  those  locally  caused ;  that  is,  by  direct  action 
upon  the  cerebellum  or  the  vestibular  apparatus  (compare  Lecture  XX). 
Onlv  in  the  last  instance  does  rotary  vertigo  proper,  eventually  with  constant 
direction  of  the  turning,  usually  occur,  while  in  brain  tumors  located  else- 
where, diffuse  and  less  characteristic  distui'bances  of  the  space  consciousness 
are  more  frequent. 

Of  the  greatest  value  for  the  diagnosis  of  the  space-reducing  brain  tumors 
is  "papilloedema,"  whose  pathogenesis  has  been  made  plain  to  us  by  the  ophthal- 
mologists Schmidt  and  Manz.  The  optic  nerve  possesses  a  connective  tissue 
sheatH  whose  cavity  communicates  with  the  subarachnoid  space  of  the  brain. 
In  increasing  brain  pressure  the  cerebrospinal  fluid  consequently  presses 
against  the  "lamina  cribrosa"  of  the  optic  nerve  head  which  is  pushed  for- 
ward and  rendered  oedematous.  In  this,  also,  there  is  strangulation  of  the 
central  vein  of  the  retina,  which  favors  stasis  and  serous  infiltration.  Fig.  84 
shows  you  the  ophthalmoscopic  picture  of  such  a  papilloedema  in  a  case  of 
cerebellar  sarcoma.  The  papilla  loses  its  sharp  outline  and  appears  enlarged ; 
it  protrudes  knoblike  toward  the  vitreous,  and  is  occupied  by  a  fine  radial 
streaking  which  extends  into  the  neighboring  retina ;  its  color  is  gray-white, 
the  "physiological  excavation"  is  not  sharp,  the  vessels  are  in  places  "veiled" 


BRAIN    TUMOR 


291 


by  the  oedema,  and  altered  in  their  caliber,  the  veins  dilated,  the  arteries  nar- 
rowed; the  fine  dots  of  the  lamina  cribrosa  are  invisible.  If  the  brain  pres- 
sure disappears,  even  a  high  degree  of  papilledema  may  entirely  pass  away ; 
its  long  continuance,  however,  leads  to  an  atrophy  of  the  optic  nerve  which  is 
incurable. 

The  influence  of  increased  intracranial  pressure  upon  the  psychical  con- 
dition of  the  patient  expresses  itself  in  the  more  advanced  stages  of  brain 
tumor  by  stupor;  the  psyche  usually  remains  intact  only  in  certain  basal 
tumors  or  in  those  of  the  cerebellar  region.      Slight  confusion  manifests  itself 


Fig.  84. 
Papilledema*     Sarcoma  of  the  Left  Cerebellar  Hemisphere. 

in  that  the  patients,  when  questioned,  must  visibly  "pull  themselves  together" 
in  order  to  answer,  make  a  somnolent  impression  on  speaking,  and  left  to 
themselves,  are  entirely  apathetic  with  regard  to  the  happenings  in  the  exter- 
nal world.  Higher  degrees  of  confusion  are  accompanied  by  somnolence,  so 
that  the  patient,  even  when  taking  nourishment,  can  scarcely  be  kept  awake; 
in  the  late  stages  this  torpor  pksses  finally  into  complete  coma,  with  involun- 
tary discharge  of  faeces  and  urine.  Along  with  these  more  or  less  permanent 
disturbances  of  consciousness,  paroxysmal  attacks  of  loss  of  consciousness  also 
occur.  These  sometimes  fake  on  an  epileptiform  character  through  accom- 
panying spasms,  or  if  the  loss  of  consciousness  is  not  complete,  a  h vsteriform. 
Anomalies  of  pulse  and  respiration  in  brain  tumors  usually  only  appear 
in  advanced  stages.     As  "pressure  pulse."  is  denominated  a  pulse  slowed   to 

about  45  to  55  beats  a  minute,  but   full  and   tense;  only  exceptionally   lias   still 


292 


LECTURE     XIX 


more  marked  bradycardia  (even  20  beats  to  the  minute)  been  observed.  In 
the  terminal  stage,  in  the  place  of  bradycardia,  a  tachycardia  with  arhyth- 
mia  ("paralytic  pulse")  occurs.  That  in  tumors  which  are  located  in  the 
neighborhood  of  the  bulbar  vagus  centers,  the  pulse  anomalies  appear  earlier 
is  easily  understandable.  The  same  remark  applies  to  respiratory  disturb- 
ances which  in  general  also  appear  as  late  symptoms  only ;  slowing  and  deep- 
ening of  the  breathing,  finally  the  so-called  "Cheyne-StoJces"  respiration.  This 
is,  as  we  know,  a  type  of  breathing  characterized  by  alternating  periods  of 


Motor 


Sensibli 


A  =  Auditory. 

V  =  "Visual  Cortical  Field. 

S  C  =  Sensory  Speech  Center  (only  Left). 

a  =  Center  for  the  Foot. 


and  Sensory  I— 


yffilC°r 


:  Elbow. 
:  Hand. 
:  Finger. 


e  =  Shoulder. 

f  =  Upper  Facial. 
g  =  Lower  Facial. 


deepness  and  shallowness  of  the  breathing  with  regular  interpolation  of  an 
apnoeic  stage,  which  occurs  also  in  other  disease  conditions  (namely  in  uremia). 
A  direct  measurement  of  the  brain  pressure  is  permitted  us  by  lunibar 
puncture  (see  above,  page  253),  in  which  a  water  manometer  attached  to  a 
cannula  mounts  higher  than  normally.  If  namely  in  healthy  people  the  mano- 
metric  pressure  measured  while  lying  down  is  usually  from  -10  to  150  mm 
water,  in  cerebral  tumor  it  usually  runs  from  250  mm  to  900  mm.  We  can 
generally,  however,  dispense  with  exact  measurements  and  satisfy  ourselves 
with  determining  that  in  intracranial  hypertension  the  cerebrospinal  fluid, 
instead  of  being  discharged  in  drops,  spurts  from  the  cannula  in  a  stream  as 
soon  as  we  withdraw  the  mandrin.  Absolutely  to  be  avoided  is  lumbar  punc- 
ture in  all  cases  in  which  there  is  a  possibility  of  a  tumor  being  located  in 
the  posterior  fossa  of  the  skull ;  since  in  this  eventuality  sudden  death  from 


BRAIN    TUMOR 


293 


the  effect  of  rapid  decompression  upon  the  vital  centers  of  the  medulla  is 
risked.  Also  in  tumors  located  in  the  frontal  lobe,  as  soon  as  the  raise  of 
pressure  within  the  skull  has  reached  a  great  height,  lumbar  puncture  is  not 
without  danger.  Its  diagnostic  application  is  hence  to  be  limited  as  much 
as  possible  in  brain  tumors.  This  last  remark  applies  with  even  more  force 
to  brain  puncture  after  Neisser  and  Pollack.  As  de  Quervain  very  properly 
emphasizes,  this  measure  is  too  dangerous  and  in  its  results  too  uncertain  to 
be  properly  reckoned  among  the  diagnostic  aids  of  the  physician;  on  the 
other  hand,  it  can  occasionally  be  a  valuable  aid  to  the  surgeon  when  every- 
thing is  prepared  for  operation. 

Xow  as  to  the  focal  symptoms  of  brain  tumor,  the  accurate  determination 
of  the  seat  of  the  tumor,  as  deduced   from   the  knowledge  of  them,  is   often 

Cortical  Field. 


Motor  M 


ensible  )  |  and  Sensory  t.r-rVl 


— ~-^-m   Cortical  Fields. 


V  =  VisuaL    0  =  Olfactory  Cortical  Field.     FUr  Hufte=For  Hip. 


B  difficult  and  responsible  bask.  It  is  impossible  for  me,  within  the  limits  of 
these  lectures,  to  enter  into  the  particularities  with  regard  to  methods  of 
localization  which  you  will  find  assembled  in  my  "Compendium  of  Topical 
Brain  and  Spinal  Cord  Diagnosis."  Only  the  most  important  points  will  be 
here  considered. 

Tumors  of  the  motor  region  of  the  cortex  lead,  in  the  majority  of  cases, 
to  the  so-called  "cerebral  monospasms"  as  well  as  to  "cortical  {Jaclcsoman 
epileptic  attacks"  (both  representing  cortical  irritative  symptoms),  on  the 
other  hand,  also,  to  "cortical  paralyses"  (to  he  considered  as  defect  symptoms 
in  consequence  of  destruction  of  cortical  motor  centers).  Figs.  85  and  S(> 
will  show  you  the  distribution  of  these  centers.  As  cerebral  monospasm  we 
designate  tonic-clonic  spasms  which  affect  a  muscular  center  up  to  this  time 
normal  or  already  paretic  (face,  arm,  hand,  etc.),  and  leave  behind  them 
permanent  paresis,  or  a  permanent  increase  of  an  already  present  paresis. 
Jacksonian   or   cortical   epileptic   at  lacks  begin   usually   as   monospasm,   and. 


294  LECTURE    XIX 

as  a  rule,  tonically.  The  contractions,  however,  do  not  remain  limited  to 
their  starting-point,  as  does  an  ordinary  cortical  monospasm  (to  the  muscles 
about  the  mouth,  for  instance),  but  they  extend  to  the  arm,  and  finally,  also, 
to  the  leg  of  the  same  side  of  the  body  (naturally  the  seat  of  the  tumor  is 
on  the  side  of  the  brain  opposite  to  the  spasm).  When  the  attack  begins  in 
the  arm,  after  the  whole  arm  is  affected,  the  face,  and  then  the  leg,  become 
involved.  As  the  crural  type  is  designated  the  succession,  lower  limb,  upper 
limb,  face.  The  tumor  sets  the  pyramidal  cells  in  its  neighborhood  into  a 
condition  of  irritability  which  spreads  itself  like  waves  over  the  surface  of  the 
water,  gradually  to  the  neighboring  cortical  regions.  The  muscles,  indeed, 
are  attacked  in  an  order  which  corresponds  to  the  arrangement  next  to  one 
another  of  their  cortical  centers.  In  severe  cases,  Jacksoniun  convulsions  pass 
over  also  to  the  extremities  on  the  other  side  and  can  be  accompanied  by  loss 
of  consciousness.  A  very  intense  irritation  can  also  propagate  itself  through 
the  commissural  tracts  of  the  corpus  callosum,  etc.,  to  the  cortical  motor 
field  of  the  other  hemisphere,  and  can  finally  benumb  the  centers  for  the  higher 
psychical  functions  (in  the  first  place  the  frontal  cortex).  Cortical  mono- 
plegia usually  develops  as  a  gradually  increasing  spastic  paresis ;  according 
to  its  extent,  we  speak  of  monoplegia  cruralis,  brachialis,  facialis,  facio- 
brachialis,  facio-lingualis.  Facial  paralysis  is  limited  to  the  lower  division 
of  the  muscles,  since  the  upper  is  innervated  from  both  hemispheres  so  that 
the  loss  of  one-sided  innervation  is  comparatively  unimportant  clinically. 

While  on  account  of  their  surgical  interest  the  tumors  of  the  cortical 
motor  region  a  short  time  ago  stood  so  much  in  the  foreground  of  all  brain 
tumors  that  v.  Bcrgmann  defined  brain  surgery  as  the  "surgery  of  the  central 
convolutions,1'  to-day  the  tumors  of  the  posterior  fossa  of  the  skull  are  also 
relatively  frequently  operated  upon.  The  description  of  their  symptomatology 
we  will  reserve  for  the  next  lecture  devoted  to  cerebellar  affections.  Also  the 
most  important  facts  with  regard  to  the  not  very  rare  tumors  of  the  hypo- 
physis of  late  equally  attacked  by  the  surgeons,  we  will  put  off  until  a  later 
lecture  (XXIV). 

The  remaining  localizations  of  intracranial  growths  indicate  usually  in- 
operability ;  the  knowledge  of  their  special  clinical  symptom-complexes  is 
hence  somewhat  less  important,  and  we  can  content  ourselves  here  with  a 
comprehensive  synopsis. 

Tumors  of  the  frontal  lobe  often  produce  a  marked  defective  intelligence, 
somewhat  like  that  of  progressive  paralysis ;  again,  the  so-called  "Witzelsucht" 
("moria") — a  tendency  to  silly  jests  with  loss  of  ethical  feelings  and  pleasure 
in  offensive  demeanor.  In  affection  of  the  lowest  frontal  convolution  on  the 
left  side,  that  is,  of  Broca's  center,  motor  aphasia  occurs,  as  already  men- 
tioned in  Lecture  XVIII.  Occasionally  paralysis  or  contracture  of  the  trunk 
and  neck  muscles  can  be  observed.  Tumors  on  the  lower  surface  of  the 
frontal  lobe  lead,  as  a  rule,  to  protrusion  of  the  eyeball,  to  anosmia  and  to 
an  atrophy  of  the  optic  nerve  which  often  progresses  so  rapidly  that  a  papil- 
ledema does  not  develop.  Where,  however,  the  last  occurs,  it  is  found  not 
rarely  only  upon  one  side   (that  of  the  tumor). 

Tumors   of  the   region    of   the   optic    thalamus   set   up   lateral   hemianopsia 


BRAIN'    TUMOR  295 

and  (by  their  effect  upon  the  internal  capsule)  a  progressive  spastic  paresis 
of  the  extremities  (both  on  the  opposite  side).  Further,  the  suspension  of 
certain  '"psycho  reflexes"  have  been  described  as  a  thalamus  symptom.  On 
unintentional  laughing  and  crying  the  lower  facial  muscles  of  the  opposite 
side  remain  masklike,  stiff  and  immovable,  while  voluntarily  they  can  be  put 
in  action  in  every  manner.  Finally  are  to  be  mentioned:  A  crossed,  persistent 
hemianesthesia  (which  is  usually  much  less  marked  for  touch,  pain  and  tem- 
perature than  for  deep  sensibility)  and  exceedingly  severe  continual  pain, 
which  is  refractory  to  analgesics  and  exacerbates  from  time  to  time  ("central 
pain"),  which  is  projected  into  the  contralateral  half  of  the  body. 

Tumors  of  the  temporal  lobe  are  sometimes  accompanied  by  sensorv  apha- 
sia (see  above,  page  277),  those  of  the  occipital  lobe  by  hemianopsia  (see 
above,  page  2.59).  Also  tumors  of  parietal  situation  can  produce  half-sided 
blindness  by  destruction  of  the  optic  radiations  of  Gratiolet.  With  the  last 
localization  the  symptom-complex  of  alexia,  already  mentioned,  is  not  very 
rarely  observed,  very  frequently,  however,  the  so-called  astereognosis,  that  is, 
the  inability  to  recognize  objects  by  touch  when  the  eyes  are  closed,  although 
with  this  the  elementary  sense  perceptions  are  tolerably  preserved,  and  in 
the  "pure"  cases,  which  have  been  called  "'touch  paralysis,"  are  found  entirely 
intact. 

Characteristic  of  tumors  of  the  corpus  callosum  are:  Apraxia  (see  above, 
page  283),  bilateral  hemiparesis  without  increase  of  reflexes,  and  without 
Babinski's  symptom.  According  to  the  more  or  less  median  situation  of  the 
lesion,  the  intensity  of  the  paresis  on  the  two  sides  is  even  or  uneven;  also, 
unilateral  hemiparesis  can  be  combined  with  contralateral  motor  irritative 
symptoms  (for  example,  convulsions  or  hemichorea).  In  corpus  callosum 
lesions,  sensibility,  as  a  rule,  remains  intact,  also  the  functions  of  the  cranial 
nerves;  only  in  involvement  of  the  most  anterior  portion  of  the  corpus  callosum 
the  facial  is  found  paretic.  Tumor  formation  in  the  internal  capsule  pro- 
duces :i  chronic  progressive  hemiplegia  whose  clinical  end  result  closely  approx- 
imates that  of  the  acute  form.  In  tumors  of  the  region  of  the  corpora  quad- 
rigemina,  pupillary  paralysis,  paralyses  of  the  external  eye  muscles,  ataxia, 
impaired  hearing,  visual  disturbances,  nystagmus  directed  vertically  anil  out- 
ward, are  found.  That  the  origin  of  a  tumor  pressing  upon  the  lamina  quad- 
rigemina  is  in  the  pineal  gl^gd  (epiphysis  cerebri)  is  indicated  by  the  occur- 
rence of  conditions  of  sexual  excitement,  abnormal  development  of  hair  and 
adiposity,  in  young  individuals,  also,  occurrence  of  a  hyperplasia  of  the 
genitals  and  an  abnormal  increase  of  the  length  of  the  body  (the  so-called 
"dyspinealism,"  sec  Lecture  XXIV). 

The  tumors  of  the  base  of  the  brain  manifest  themselves  in  general  by 
early  and  marked  involvement  in  the  clinical  picture  of  the  cranial  nerve 
toots,  of  irritative  symptoms,  hyperesthesia  and  neuralgiform  "root  pains" 
in  the  region  of  the  trigeminus  are  to  be  specially  mentioned.  The  irritative 
symptom  of  the  motor  trigeminus  is  trismus,  the  cramp  of  the  masseters 
leading  to  grinding  the  teeth,  that  of  the  facial  the  twitching  of  tin  face 
muscles.  Also  sensory  irritative  phenomena  {e.g.,  tinnitus  aurium)  are  not 
rare.     The  paralytic  symptoms  can  affeel  every  brain  nerve.     The}   are,  as  a 


296  LECTURE    XIX 

rule,  multiple,  but,  on  the  other  hand,  usually  develop  only  on  one  side.  Most 
frequently  affected  are  the  facial,  abducens,  and  oculo-motorius  whose  altera- 
tion, as  a  rule,  first  manifests  itself  by  ptosis.  Destruction  of  the  pyramids 
is  quite  rare;  slighter  affection  comes  only  little  by  little  into  plain  view — 
through  the  Babinski  reflex,  spasticity,  etc.  Only  lesions  in  the  neighbor- 
hood of  the  crus  cerebri  have  a  great  tendency  to  destroy  the  cortico-spinal 
tracts,  so  that  in  this  location  of  the  tumor  the  Weber  symptom-complex 
hemiplegia  alternans  oculomotoria  (see  page  270)  comes  under  observation. 
Papilloedema  usually  occurs  early.  If  the  tumor  is  located  in  the  caudal  divi- 
sion of  the  base  of  the  brain,  bulbar  paralytic  symptoms  arise  (see  page  120). 

Prognosis  and  Treatment 

The  prognosis  of  brain  tumor  is  in  general  a  very  gloomy  one.  Sponta- 
neous improvement  and  recovery  has  been  observed  in  aneurisms  (obliteration), 
in  cysts  and  serous  meningitis  (resorption),  in  tubercle,  cysticercus,  echino- 
coccus  (calcification),  in  gumma  (regression)  ;  the  process  of  cure  may  be 
hastened  in  syphilitic  new  formations  by  anti-syphilitic  treatment,  also  mercury 
and  iodide  cures  have  been  of  benefit  in  serous  meningitis.  In  true  neoplasms 
there  are  cases  in  which  in  benign  tumors  recovery  up  to  a  certain  point  has 
occurred  through  these  growths  undergoing  calcification  or  ossification,  very 
great  rarities,  so  that  death  in  coma  or  from  rupture  of  a  vessel  is  the  nearly 
regular  ending  of  the  frightful  disease  picture  (although  sometimes  it  is  only 
slowly  progressive,  and  eventually  interrupted  by  stationary  periods).  In 
most  cases,  hence,  operative  interference,  in  spite  of  the  very  great  dangers 
which  it  presents  in  many  instances,  offers  to  us  the  only  therapeutic  way 
standing  open. 

In  principle,  curable  by  operation  are,  properly,  only  tumors  on  the 
cerebral  convexity  and  upon  the  cerebellum  (provided  that  they  are  cystic, 
or,  if  solid,  are  well  limited  and  can  be  extirpated  in  toto*);  perhaps,  also, 
hypophysis  tumor.  Very  many  cases  can,  however,  derive  great  benefit  from 
a  pidliative  operation ;  this  is  the  so-called  "decompressive  craniectomy." 

With  regard  to  the  indications  for  surgical  interference,  I  would  impress 
upon  you  the  following:  In  too  many  cases  decision  as  to  an  operation  is  put 
off  for  a  long  time  to  try  combined  mercui'y  and  iodide  treatment  (which 
usually  has  also  in  glioma  and  sarcoma  a  decided  but  unfortunately  only 
temporary  effect — probably  on  account  of  resorption  of  the  secondary  ven- 
tricular dropsy)  until  it  finally  proves  itself  necessary.  Such  treatment  is 
usually  ordered  even  in  the  absence  of  a  history  of  syphilis,  while  on  the 
other  hand  it  should  not  be  forgotten  that  former  syphilitics  also  can  have 
non-luetic  brain  affections.  It  is  always  to  be  remembered  that  direct  danger 
to  life,  unbearable  intensity  of  the  headache,  threatened  loss  of  vision  or  de- 
mentia, present  pressing  indications  for  decompressive  craniectomy.  Babinski 
has  properly  emphasized  that  this  indication  should  be  accepted  much  more 

*  Gliomata  show  such  indefinite  limitation  from  the  healthy  surrounding  tissue  and  such  a 
gradual  passage  over  into  it,  that  their  operative  prognosis,  in  spite  of  their  pathologico-ana- 
tomical  benignness,  is  unfavorable. 


BRAIN    ABSCESS  297 

frequently  than  it  generally  is.  Even  in  malignant  neoplasms  it  can  act  pal- 
liatively  ;  in  benign  tumors  inaccessible  to  extirpation,  which,  however,  are 
capable  of  coming  to  a  standstill  in  growth,  it  may  prevent  the  occurrence 
of  irreparable  destruction;  in  serous  meningitis  it  may  be  directly  curative. 
Where  the  brain  pressure  is  immediately  threatening  it  may  be  necessary  to 
advise  first  operative  decompression,  and  then  a  mercury  cure.  In  tumors 
not  exactly  localizable,  temporal  craniectomy  recommends  itself,  since  the 
muscles  of  mastication  oppose  in  suitable  manner  the  occurrence  of  brain 
prolapse.  If  the  disease  is  located  in  the  posterior  fossa,  the  occipital  region 
is,  as  a  rule,  the  place  of  choice.  The  neck  muscles  also  affect  a  good  closure. 
Less  favorable  conditions  in  this  respect  is  afforded  by  parietal  craniectomy 
which  is  undertaken  for  tumors  of  the  motor  region. 

B.    Brain  Abscess 

The  infection  lying  at  the  base  of  brain  abscess  usually  enters  the  organ 
by  continuity,  more  rarely  by  metastasis.  The  first  applies  to  traumatic 
and  otitic  brain  abscesses  which  are  observed  after  septic  wounds  of  the  skull 
and  suppuration  in  the  ear;  in  these  the  germs  pass  by  the  lymph  channels, 
along  the  nerve  sheaths,  or  by  means  of  septic  venous  thromboses  into  the 
brain  substance.  Metastatic  infections  reaching  the  brain  by  way  of  the 
blood  vessels,  namely,  after  lung  abscesses,  empyemata  of  the  pleura  and 
putrid  bronchitis,  lead  to  cerebral  pus  collections.  Brain  abscesses  can  be 
multiple  or  solitary;  in  the  last  case  they  sometimes  reach  the  size  of  an  apple. 
Their  pus  contains  staphylococci,  streptococci,  colon  bacteria,  different  anae- 
robic germs,  etc.  Otitic  abscesses  locate  by  preference  in  the  temporal  lobe 
or  in  the  cerebellum ;  traumatic  or  metastatic,  naturally  show  a  very  variable 
localization. 

The  clinical  symptoms  of  brain  abscess,  like  those  of  brain  tumor,  may 
be  divided  into  general  symptoms  and  focal  symptoms.  In  regard  to  the 
last,  there  is  entire  agreement  between  the  two  diseases,  and  also  the  general 
Symptoms  show  very  great  analogy.  Only  papilloedema  is  much  rarer  in 
abscesses  than  in  brain  tumors;  in  them  fever,  which  sometimes  has  a  septic 
character  (with  rapid  rise  of  temperature  and  chills),  sometimes,  however, 
expresses  itself  only  in  moderate  evening  rises  of  temperature,  plays  a  de- 
cidedly  greater  role  That  docs  not  mean  indeed,  that  eases  with  entirely 
afebrile  course  are  great  rarities;  on  the  contrary,  they  represent  a  very 
considerable  minority  which  may  well  depend  upon  a  relatively  frequent  for 
mation  of  thick  "abscess  membrane"  which  prevents  the  resorption  of  the 
septic  substances.  The  encapsulation  can  go  so  far  thai  the  abscess  he 
Comes  "latent,"  and  either  causes  no  longer  any  difficulties  or  manifests  itself 
Only  through  the  focal  symptoms  which  have  become  stationary.  Such  a  be- 
coming latent,  however,  cannot  he  considered  equivalenl  to  a  cure,  as  even 
after  latency  for  years  acute  lighting  up  again  of  the  process  -till  threatens. 

On  tliis  account  the  treatment  of  brain  abscess  (in  SO  far  as  it  can  lie  diag- 
BOSed   and   an   operatively   accessible   point    can   he   localized)    in    general   can   he 

tuitablv  and  conclusively  summed  up  in  the  old  surgical  axiom,  "Where  there 


298  LECTURE    XIX 

is  pus,  evacuate."  The  diagnosis,  however,  in  those  cases  in  which  a  skull 
trauma,  suppuration  in  the  ear  or  in  the  mastoid,  a  lung  abscess,  etc.,  cannot 
be  recognized,  and  where  fever  and  chills  are  absent,  is  mostly  exceedingly 
difficult  with  regard  to  its  distinction  from  brain  tumor;  indeed,  sometimes  an 
impossibility  even  for  the  experienced  neurologist,  or  it  must  be  made  by 
exploratory  trephining  and  puncture  of  the  brain.  Occasionally  the  differ- 
ential diagnosis  from  purulent  meningitis,  upon  the  description  of  which  we 
will  immediately  enter,  is  difficult. 

C.  Purulent  Cerebral  Meningitis 

What  we  have  said  as  to  the  etiological  factors  in  brain  abscess  applies 
also  to  purulent  meningitis.  It  can  also  arise  from  traumatic,  otitic  or  metas- 
tatic causes.  The  last  method  of  origin,  however,  is  observed  here  much  more 
frequently  than  in  brain  abscess ;  along  with  the  affections  of  the  lung  and 
pleura  mentioned,  in  purulent  meningitis,  ulcerous  endocarditis,  puerperal  in- 
fection, further  typhoid,  pneumonia,  influenza,  etc.,  play  a  not  inconsiderable 
role.  A  relatively  frequent  manner  of  meningeal  infection  by  continuity  is 
further  the  progressive  infection  of  the  so-called  emissary  veins  of  the  skull- 
cap and  the  lymph  vessels  in  erysipelas.  Other  important  etiological  factors 
are,  finally,  the  septic  diseases  of  the  orbit,  the  nose,  the  frontal  sinuses,  and 
the  ethmoid  cavities,  as  well  as  the  rupture  of  a  brain  abscess  into  the  sub- 
arachnoid space. 

The  often  exceedingly  voluminous  pus  collection  in  cerebral  meningitis 
usually  affects  the  convexity  of  the  brain  in  such  preponderant  fashion  that 
it  is  often  directly  denominated  '•meningitis  of  the  convexity.'' 

The  clinical  picture  of  this  affection  shows  so  great  a  similarity  to  that 
of  epidemic  meningitis  that,  referring  to  Lecture  XVI,  we  can  dispense  with 
a  thorough  description  of  the  individual  symptoms  and  content  ourselves 
with  a  summary  sketch  of  its  symptomatology.  The  initial  symptoms  of 
purulent  meningitis  are  very  often  veiled  by  those  of  the  underlying  disease, 
for  instance,  of  erysipelas  or  of  purulent  otitis.  Where,  however,  they  have 
not  developed  from  such  a  primary  infection,  they  are  usually  characterized 
by  quite  masked  and  sudden  beginning. 

Fever  is  present  in  by  far  the  majority  of  cases  and  of  rather  continued 
character.  As  a  rule,  the  temperature  varies  between.38°  and  -K)°  C.  (100.4 
and  104J  F.)  ;  apart  from  the  initial  chill  sometimes  occurring,  many  patients 
in  the  further  course  of  the  disease  have  marked  shivering  from  time  to  time. 
Headaches  of  terrible  severity  and  diffuse  distribution  are  present  from  the 
start,  and  show  themselves  even  in  half  coma  by  the  patient  frequently  put- 
ting his  hand  to  his  head.  The  pulse  is  rapid,  respiration  hurried.  Cerebral 
vomiting  is  only  frequent  in  the  initial  stages.  Stiffness  of  the  neck  is  some- 
times developed  quite  early,  also  other  conditions  of  muscular  tension ;  boat- 
shaped  retraction  of  the  abdomen,  trismus,  grimacing,  contractures  in  flexion 
of  the  extremities,  Kernig's  symptom.  On  the  other  hand,  general  convul- 
sions coming  on  in  attacks,  are  rare,  more  frequent,  localized  twitching  of 
one  extremity,  one-half  of  the  face,  etc.     The  pupils  are  at  the  start  narrowed 


CEREBRAL    MENINGITIS  299 

and  frequently  unequal;  in  otitic  meningitis  the  myosis  is  more  marked  on 
the  side  of  the  affected  ear;  in  the  terminal  stage  they  are  wide.  Of  other 
ocular  symptoms,  there  occur  occasionally  convergent  and  divergent  strabis- 
mus, as  well  as  tonic  spasm  of  the  orbicularis  palpebrarum.  Further  disease 
signs  are,  hyperesthesia  of  the  skin,  the  so-called  "taches  cerebrales"  of 
Trousseau  (see  Lecture  XVI,  page  250),  retention  of  urine  and  faces,  par- 
ticularly regularly,  however,  deliria,  which  manifest  themselves  sometimes  by 
the  murmuring  of  incoherent  words  as  well  as  by  disorientation  for  time  and 
place,  sometimes,  however,  by  screaming,  motor  jactitation,  even  furibund  con- 
ditions.    IKic  and  there  papilloedema,  usually  of  moderate  grade,  occurs. 

The  disease  results  fatally  in  the  great  majority  of  cases.  In  the  terminal 
stage  the  clinical  picture  changes,  in  that,  in  the  place  of  contractures,  par- 
alvses  (monoplegia,  hemiplegia,  paraplegia,  even  tetraplegia),  in  the  place  of 
delirium,  a  torpor  deepening  to  coma;  instead  of  myosis,  mydriasis:  instead 
of  retention,  incontinence  of  stools  and  urine,  instead  of  quickening  of  the 
pulse  and  respiration,  their  slowing  occur,  and  finally  death  from  respiratory 
and  cardiac  paralysis  follows.  Occasionally  at  the  last,  Cheyne-Stokcs  breath- 
ing is  observed. 

Cases  going  on  to  "recovery"  almost  always  leave  behind  serious  defects, 
as  deafness,  mental  weakness,  idiocy,  etc. 

The  treatment  agrees  in  general  with  that  of  epidemic  meningitis.  Natu- 
rally, upon  the  beginning  of  meningeal  symptoms,  the  underlying  disease  must 
be  searched  for,  and  energetically  combated;  for  example,  the  mastoid  tre- 
phined, the  frontal  sinus  opened,  furuncle  of  the  face  incised,  etc.  Repeated 
lumbar  {junctures  are  always  in  place,  although  they  only  rarely  produce 
tasting  effect;  nevertheless,  they  quite  frequently  affect  considerable  alleviation, 
especially  as  to  headache  and  delirium.  When  the  puncture  fluid,  at  first 
cloudy,  later  distinctly  purulent,  becomes  by  degrees  again  free  from  leucocytes 
and  clearer,  this  is  naturally  favorable  prognostically.  Definite  surgical  treat- 
ment (trephining,  opening  the  dura,  washing  out,  drainage)  has  of  late  been 
recommended  by  Girard  and  others;  the  results  are,  however,  unfortunately 
very  uncertain,  as  a  rule.     For  the  rest,  see  Lecture  XVI,  page  25L 

D.      Tuberculous  Meningitis 

A  special  clinical  and  anatomical  consideration  is  due.  to  the  by  far  must 
frequent  variety  of  metastatic  inflammation  of  the  pia-arachnoid,  tuberculous 
meningitis,  which  occurs,  as  a  rule,  in  young  individuals  affected  by  lung,  bone 
or  joint  tuberculosis.  It  is  particularly  often,  also,  a  symptom  in  acute  miliary 
tuberculosis.  Tuberculous  meningitis  arising  by  continuity  is  much  behind 
Hi.  metastatic  form  in  importance  and  frequency;  tuberculous  caries  <>(  the 
skull  bones,  ear  tuberculosis,  solitary  tubercle  of  the  brain,  etc.,  usually  fur- 
or.I:  a  starting  point  far  it.  As  exciting  cause-,,  injuries  to  the  head  (evc;i 
bloodless  ones),  insolation,  taking  cold,  overexertion,  as  well  as  psychical  exciti 
in. hi.  come  into  consideration.  So.  I  have  observed  in  a  girl  with  slighl  affee 
Bon  of  the  lung  apices,  a  tuberculous  meningitis  terminating  fatally  and  con- 


300  LECTURE    XIX 

firmed  at  the  autopsy,  which  occurred  in  immediate  connection  with  a  violent 
quarrel  with  her  unfaithful  lover,  as  acute  delirium,  and  which  was  at  first 
quite  pardonably  interpreted  as  hysteria  by  the  family  physician.  In  con- 
tradistinction to  purulent  meningitis,  tuberculous  meningitis  in  the  great  ma- 
jority of  cases  spreads  itself  over  the  region  of  the  base  of  the  brain.  It  is 
hence  designated  as  basilar  meningitis.  The  extension  of  the  inflammatory 
process  into  the  fissure  of  Sylvius  is  almost  the  rule,  while  the  convexity  of 
the  brain  is  usually  but  inconsiderably  affected.  In  the  region  of  the  disease, 
an  opalescent  or  cloudy  "gelatinous"  infiltration  of  the  pia  and  arachnoid  is 
found ;  the  fluid  is  seropurulent  only  in  places.  At  most  varied  points,  how- 
ever, particularly  in  the  neighborhood  of  the  vessels,  a  more  or  less  abundant 
deposit  of  miliary,  whitish,  tubercular  nodules  is  noted,  which  anatomically 
present  the  well-known  structure  of  tuberculous  granulations  (giant  cells, 
etc.).     Also  Koch's  bacillus  can  be  recognized  in  most  cases. 

The  disease  picture  of  tuberculous  meningitis  has  so  great  similarity  to 
that  of  purulent  meningitis  that  in  order  to  avoid  repetition,  I  will  limit  my- 
self more  or  less  to  emphasizing  the  differences  in  its  course  and  symptoma- 
tology. 

Only  exceptionally  does  tuberculous  meningitis  begin  suddenly.  In  the 
great  majority  of  cases  there  is  a  definite  prodromal  stage  of  from  one  to 
several  weeks.  The  patient  is  peevish  and  irritable,  sometimes  also  apathetic 
and  soporous.  In  many  cases  the  alternating  redness  and  pallor  of  his  face 
is  striking  to  those  surrounding  him.  He  loses  all  appetite  and  emaciates 
rapidly ;  usually  there  is  obstinate  constipation,  somewhat  less  frequently, 
nausea  or  vomiting.  Soon  there  are  irregular  rises  of  temperature  with  slight 
shivering,  further,  headache,  stiffness  of  the  neck,  confusion  at  night — and  so 
it  proceeds  finally  to  the  completely  developed  meningitic  symptom-complex, 
in  which  headache,  cerebral  vomiting,  stiffness  of  the  neck,  and  tension  in  other 
muscles,  vasomotor  disturbances,  hyperesthesia  of  skin  and  muscles,  Kernlg's 
and  Leichcnstern's  signs  (see  Lecture  XVI,  page  24-9),  reach  a  very  high 
degree.  In  children  opisthotonus  is  so  excessively  developed  that  the  occiput 
almost  rests  upon  the  back ;  there  is  frequently  also  very  great  contracture  in 
flexion  of  the  lower  extremities.  The  delirium  manifests  itself  usually  in  rest- 
less grasping  movements  of  the  hands,  muttering,  etc.  ("muttering  delirium"). 
In  contradistinction  to  purulent  meningitis,  the  relatively  slight  elevation  of 
temperature  (usually  between  38°  and  39°  C),  is  striking;  frequent,  also,  is  the 
absence  of  quickening  of  the  pulse,  if  not  its  slowing.  Lumbar  puncture  shows 
in  the  initial  stages  almost  always  a  lymphocytosis  of  the  spinal  fluid,  further 
on  in  its  course,  however,  the  leucocytes  may  preponderate  just  as  much  as 
in  purulent  meningitis.  Naturally,  a  positive  finding  of  tubercle  bacilli  in  the 
spinal  fluid  is  decisive.  As  a  criterion  of  the  basal  localization  is,  further,  the 
much  greater  frequency  of  cranial  nerve  symptoms  important :  paralyses  of 
the  abducens,  oculomotorius,  facialis,  deafness,  optic  neuritis.  Symptoms  from 
the  side  of  the  pyramidal  tracts  (Babinski  and  Oppenheim's  reflexes,  ankle 
clonus)  not  rarely  occur.  The  ophthalmoscopic  finding  of  tubercles  in  the 
choroid  is,  on  the  other  hand,  a  very  great  rarity  and  scarcely  ever  available 
for  confirming  the  etiological  diagnosis   (for  which  in  children  the  v.  Pirqua 


CEREBRAL    MENINGITIS  301 

cutaneous  reaction  may  be  called  into  aid,  in  case  the  primary  tuberculous 
focus  is  latent). 

The  prognosis  is  a  very  bad  one.  Of  the  bacteriologically  confirmed  ob- 
servations of  tuberculous  meningitis,  only  very  exceptionally  have  cases  sur- 
vived the  disease,  and  here  even  the  recovery  was  usually  with  defect ;  also 
some  of  these  patients  perished  later  with  recurrence.  The  treatment  agrees 
in  general  with  that  of  other  meningitides. 

E.     Internal  Hemorrhagic  Pachymeningitis 

In  the  etiology  of  this  disease,  chronic  alcoholism  occupies  the  first  place 
in  frequency  and  practical  importance;  besides,  it  occurs  in  different  disease 
conditions  accompanied  by  the  "hemorrhagic  diathesis"  (scurvy,  leucemia, 
hemophilia,  pernicious  anemia,  etc.),  as  well  as  after  the  most  varied  ex- 
hausting infectious  diseases.  Exciting  causes  for  this  disease,  which  affects 
liv  preference  those  of  advanced  age  and  the  male  sex,  are,  above  everything 
else,  contusions  and  wounds  of  the  head.  For  the  rest,  many  cases  remain 
etiologically  entirely  unexplained.  According  to  Obersteincr,  hemorrhagic 
pachymeningitis  occurs  in  about  20  per  cent,  of  paretics  as  a  complication 
of  the  end   stage. 

Anatomically,  internal  hemorrhagic  pachymeningitis  presents  itself  as  fol- 
lows: There  arise  upon  the  inside  of  the  dura  mater,  flat,  membrane-like  de- 
posits, extraordinarily  rich  in  vessels,  of  inflammatory  granulation  tissue, 
which  later  changes  into  connective  tissue.  The  walls  of  these  vessels  show 
more  or  less  marked  evidence  of  degeneration  and  a  great  tendency  to  rupture, 
wliich  have  as  a  result  the  formation  of  blood  coagula  upon  the  membrane  cov- 
ering the  dura.  New  granulation  tissue  develops  in  these  coagula,  and  so  there 
arises  from  hemorrhages  which  later  show  themselves  only  as  brown  pigment, 
Strata  layer  built  upon  layer.  This  hemorrhagic  pachymeningitis  develops 
(usually  bi-laterally,  although,  as  a  rule,  asymmetric)  particularly  over  the 
convexity  of  the  hemispheres,  namely  over  the  parietal  lobes;  il  is  more  rarely 

found    at    the   base   of   the    skull. 

The  disease  iii  slight  cases  can  run  its  course  without  symptoms  and  is  then 
counted  among  the  so-called  "accidental  autopsy  findings."'  This  applies,  for 
example,  to  most  of  the  eases  found  in  paretics.  The  more  severe  eases,  on 
the  contrary,  produce  grave  symptoms,  which  indeed  have  in  themselves  little 
typical,  and  are  subjected  to  such  great  variations  in  their  character  and 
grouping  that  confusion  with  leptomeningitis,  brain  tumor,  hemorrhages,  etc., 
is  very  frequent.  In  the  early  stages  severe  headache  is  the  chief  symptom. 
Since  the  patient  besides  this  usually  complains  of  vertigo,  shows  different 
speech  disturbances  and  defects»of  memory  and  in  his  movements  can  give 
evidence  to  a  certain  slowness,  weakness  and  cl siness,  we  are  greatly  reminded 

of  arteriosclerosis  of  the  brain,  cerebral  syphilis  or  dementia  paralytica.  Also 
Conditions  of  psychical  excitement  and  epileptiform  attacks  are  described. 
Later  the  condition  is  complicated  by  suddenly  occurring  (probably  corre- 
sponding to  further  hemorrhage)  focal  symptoms  which  sometimes  disappear 
again,   sometimes    persist:     Monoplegias,   hemiplegias,   also    tetraplegias,   con- 


302  LECTURE    XIX 

jugate  deviation  of  the  eyes  and  head,  sometimes  also  spasms  in  the  individual 
extremities  or  in  the  face.  Stiff  neck  is  rare,  Kernig's  symptom  somewhat 
more  frequent.  The  pupils  are  usually  narrow,  sometimes  unequal,  and  react 
slowly ;  the  tendon  reflexes  are  usually  exaggerated,  ankle  clonus  and  Babinski, 
on  the  other  hand,  but  rarely  present.  There  can  be  also  a  weakening  and 
diminution  of  the  reflexes.  The  intelligence  diminishes  rapidly.  In  cases  run- 
ning a  fatal  course,  the  symptom  complex  of  brain  pressure  is  gradually  es- 
tablished (slowing  of  the  pulse,  papilledema,  etc.)  and  the  patient  dies  in  coma. 
A  standstill  or  partial  regression  of  the  disease  picture  can  occur  in  cases 
which  have  not  progressed  too  far.  Along  with  treatment  of  the  underlying 
disease,  in  all  cases  an  energetic  iodide  cure  should  be  tried.  To  be  recom- 
mended further,  are  free  purgation,  bland  diet,  abstinence  from  alcohol  and 
from  smoking.  Apoplectiform  incidents  are  to  be  treated  after  the  principles 
laid  down  in  Lecture  XVII. 

F.     Thrombosis  of  the  Brain  Sinuses 

The  thrombotic  occlusion  of  the  different  brain  sinuses  (particularly  of 
the  transverse,  superior  longitudinal  and  cavernous  sinuses)  can  arise  autoch- 
thonously  or  secondarily,  that  is  by  the  extension  of  infection  or  through 
metastasis.  In  the  first  instance  there  is  the  so-called  "marantic"  thrombosis 
which  is  observed  in  deci'epit  individuals,  in  chlorosis,  pernicious  anemia,  fol- 
lowing exhausting  infectious  diseases,  particularly,  however,  in  the  "atrophy 
of  nurslings."  Secondary  or  infectious  sinus  thrombosis  arises  in  consequence 
of  erysipelas,  suppurations  in  the  petrous  bone,  in  general  sepsis,  pyemia,  etc. 
The  thrombotic  occlusion  of  a  large  sinus  manifests  itself  clinically  by  severe 
headache  beginning  suddenly,  by  confusion  and  by  spasms  in  the  extremities; 
in  infectious  thromboses  these  symptoms  are  accompanied  by  chills  and  fever. 
Characteristic,  however,  are  the  local  signs  of  stasis ;  in  thrombosis  of  the 
cavernous  sinus  the  lids  are  swollen,  the  eyeballs  protrude ;  in  thrombosis  of 
the  transverse  sinus  the  neighborhood  of  the  mastoids  is  cedematous  and 
marked  by  dilated  veins ;  in  thrombosis  of  the  longitudinal  sinus,  the  temporal 
veins  are  swollen,  and  there  is  also  venous  stasis  in  the  nasal  mucous  membrane 
and  epistaxis.  Papilloedema  is  frequent  only  in  cavernous  thrombosis,  rarest 
in  transverse  thrombosis.  Extension  of  the  thrombosis  into  the  jugular  vein 
is  accessible  to  direct  palpation. 

The  prognosis  of  these  conditions  is  very  serious,  as  even  mild  sinus  throm- 
boses, if  they  reach  a  higher  degree,  produce  oedema  of  and  hemorrhages  into 
the  brain.  Onlv  clotting  of  limited  extent  is  susceptible  of  recovery,  a  result 
which  we  seek  to  assist  by  the  application  of  leeches  to  the  neck,  iodide  cures, 
etc.  Infectious  thromboses,  on  the  other  hand,  demand  operative  removal, 
which,  however,  only  in  the  minority  of  cases  is  able  to  prevent  death  from 
diffuse  meningitis. 

G.     Non-Suppurative  Encephalitis 

The  non-purulent  inflammations  of  the  brain,  if  we  ignore  the  cerebral 
localization   of  the   Hcine-Medin   disease,   and   the   inflammatory   forms   of  in- 


ENCEPHALITIS  303 

fantile  cerebral  paralysis,  are  great  rarities ;  their  knowledge  we  owe  to  Striim- 
pell,  Wernicke,  Oppenheim,  and  Cassirer.  Anatomically,  the  diseased  parts 
present  the  histological  criteria  of  inflammation  (infiltration  with  small  cells, 
dilatation  of  the  vessels,  oedematous  swelling,  degeneration  of  the  parenchy- 
matous elements,  etc.)  and  manifest  themselves  macroscopically  by  redness 
and  swelling.  Usually  there  are  also  numerous  punctiform  or  miliary  ex- 
travasations of  blood  upon  cross  section  (the  so-called  "Flea-bite  Encepha- 
litis"). 

Clinically,  different  forms  may  be  separated,  of  which  the  two  following 
are  the  most  important : 

1.     POLIOENCEPHALITIS    SUPERIOR    HEMORRHAGICA 
(WERNICKE) 

The  patients  are  taken  ill  suddenly,  with  headache,  vertigo,  vomiting,  con- 
fusion, and  somnolence,  sometimes  also  with  conditions  of  delirious  excitement, 
and  with  this  there  develops  a  rapidly  progressive,  finally  almost  total  ophthal- 
moplegia. The  gait  is  extremely  atactic.  Tremor  is  almost  the  rule,  choreic 
unrest  is  sometimes  to  be  observed.  The  disease,  which  mainly  affects  con- 
firmed alcoholics  of  middle  age,  but  can  arise  upon  the  basis  of  meat  poisoning, 
usually  leads  to  death  under  increasing  stupor  and  heart  weakness  in  one  or 
two  weeks.     Recoveries  are  rare. 

Upon  autopsy,  in  the  neighborhood  of  the  third  ventricle  and  of  the  aque- 
duct of  Sylvius,  particularly  in  the  region  of  the  eye  muscle  nuclei,  a  severe 
hemorrhagic  inflammation  is  found.* 

2.     THE  ACUTE  HEMORRHAGIC  ENCEPHALITIS  OF  ADULTS 
(STRUMPELL) 

This  is  in  the  majority  of  cases  an  accompaniment  or  the  result  of  acute 
infectious  diseases,  namely,  of  influenza.  It  can,  however,  represent  a  primary 
acute  infection.  Klieneberger  saw  it  arise  after  salvarsan  injection.  Rather 
suddenly  there  arise  with  high  fever,  headache,  unconsciousness  and  hemiplegic 
Disturbances,  which  end  in  a  few  days  in  death.  Only  a  few  cases  pass  on  to 
Recovery,  leaving  behind  more  or  less  marked  hemi-paresis.  Pathologico- 
knatomically  a  hemorrhagic  inflammatory  process  forms  the  basis  of  the  dis- 
use. This,  as  a  rule,  is  limited  to  one  hemisphere,  but  is  rather  diffusely  spread 
throughout  this  and  affects  both  the  white  ami  the  gray  matter. 

Therapeutically  we  can,  though  with  justified  pessimism,  in  both  forms,  hold 
fasl  to  the  recommendations  of  Oppenheim:  Icebag  to  the  head,  leeches  to  the 
temples  or  to  the  mastoid  region,  free  purgation  (calomel)  inunctions  of  col- 
lurgol  or  a  mercury  cure,  iodide  of  potassium,  antipyretics,  wet  packs  to  the 
body,  hot  foot  baths,  sinapisms  to  the  extremities,  etc. 

'  "Polioencephalitis  inferior  hemorrhagica,"  which  is  located  in  the  gray  matter  of  the 
medulla,  occurs  in  infectious  diseases  and  gives  the  clinical  picture  of  "acute  bulbar  paralysis." 

■  lure    VII.) 


304  LECTURE    XIX 

H.      The  Circulatory  Disturbances  of  the  Brain 

We  will  take  into  consideration  here  only  those  circulatory  disturbances 
of  the  brain  which  occur  in  the  form  of  attacks  and  rather  rapidly  disappear 
again  ;  hyperemia  of  the  brain,  anemia  of  the  brain,  and  the  so-called  concussion 
of  the  brain. 

Determination  of  blood  to  the  brain  occurring  in  attacks  forms  the  basis 
of  the  so-called  "congestion  of  the  brain"  which  occurs  under  the  influence  of 
heat,  psychical  and  especially  sexual  excitement,  etc.,  in  predisposed  individuals 
(vasomotor  neurasthenics,  arteriosclerotics,  inveterate  smokers,  drinkers,  peo- 
ple with  "true  plethora")  and  which  can  be  experimentally  produced  by  the 
inhalation  of  amyl  nitrite.  In  it,  the  face  becomes  intensely  red,  there  is  a 
feeling  of  burning  in  the  conjunctiva3,  the  carotids  and  the  temporal  arteries 
pulsate  appreciably ;  the  patient  usually  experiences  tinnitus  aurium,  a  sense 
of  pressure  in  the  head,  vertigo,  spots  before  the  eyes,  sometimes,  also,  nausea, 
becomes  excited,  sometimes,  slightly  dull  and  confused.  After  a  few  min- 
utes, or  only  after  a  half  hour  to  an  hour,  the  hyperemia  ceases  and  the  symp- 
toms disappear.  Its  danger  lies  in  arteriosclerotics,  naturally,  in  the  condition 
that  it  favors  the  occurrence  of  cerebral  hemorrhage. 

Paroxysmal  diminution  of  the  blood  in  the  brain  finds  its  clinical  expression 
in  the  temporary  reduction  of  the  brain  functions  to  the  minimum  necessary 
for  maintaining  the  vital  functions;  there  is  fainting,  syncope.  After  indefinite 
prodromal  symptoms  and  a  general  feeling  of  illness,  there  occurs,  with  small- 
ness  and  rapidity  of  the  pulse  as  well  as  great  pallor  of  the  face  and  outbreak 
of  a  cold  sweat,  often  also,  with  repeated  yawning  and  usually  decided  nausea 
(rarely  actual  vomiting),  a  condition  of  complete  muscular  relaxation  and 
marked  clouding  of  consciousness  or  even  complete  unconsciousness,  which  lasts 
for  a  half  hour  or  more,  then  to  make  way  for  the  normal  condition.  The 
prognosis  of  these  conditions  which  occur  almost  exclusively  in  anemic,  weak 
or  cachectic  persons,  is  in  general  good;  a  fatal  ending  is  only  to  be  feared  from 
syncope  after  severe  hemorrhage. 

Commotio  cerebri  represents  a  special  variety  of  circulatory  disturbance 
in  the  brain,  which  occurs  after  violent  concussions  of  the  head  in  accidents 
of  the  most  various  sorts,  and  indeed  also  without  direct  traumatization  of  the 
skull  ( for  example  in  persons  who  have  been  in  the  neighborhood  of  a  violent 
explosion).  This  "concussion  of  the  brain"  occurs  at  once  and  disappears 
after  a  short  time  (some  minutes  to  a  few  hours).  Only  very  exceptionally 
in  such  cases  in  which  the  autopsy  has  shown  an  absence  of  any  organic  injury 
of  the  brain  (for  instance,  compression  or  hemorrhage)  does  death  occur  from 
cardiac  and  respiratory  paralysis;  the  patient  in  commotio  cerebri  suddenly 
becomes  unconscious,  breathes,  as  a  rule,  shallowly  (more  rarely  deep  and 
stertorously),  has  a  small,  generally  slow  pulse,  is  pale  and  presents  coldness 
of  the  surface.  He  shows  complete  muscular  relaxation  and  does  not  react  to 
painful  stimuli  (pinching,  stabbing,  etc.).  The  pupils  are  now  narrow,  again 
dilated,  but  react  to  strong  light.  Already  during  unconsciousness,  particu- 
larly, however,  when  the  victim  comes  to  again,  there  is  frequently  retching  or 
vomiting,  and  afterward  there  persists  for  a  long  time,  headache,  staggering 


DISTURBANCES    OF    CIRCULATION"    IN    THE    BRAIN       305 

gait,  and  slight  clouding  of  consciousness.  More  severe  symptoms,  for  ex- 
ample, convulsions,  do  not  belong  within  the  limits  of  cerebral  concussion,  but 
indicate  organic  injuries  of  the  brain.  As  a  characteristic  result  cerebral  com- 
motion almost  always  leaves  behind  the  so-called  "retrograde  amnesia,"  that  is, 
the  memory  not  only  of  the  accident  itself,  but  even  the  period  (of  about  a 
quarter  of  an  hour)  preceding  it,  is  completely  obliterated  from  the  recollec- 
tion of  the  patient.  Sometimes,  however,  these  memories  return  after  a  time, 
in  which  case,  on  retrospective  diagnosis,  we  must  think  of  concussion  of  the 
brain,  when  required  to  give  an  opinion  on  the  case. 

Treatment  of  hyperemia  of  the  brain  consists  in  loosening  the  clothing 
about  the  neck,  raising  the  head,  giving  hot  foot  baths,  hot  applications  to  the 
calves,  mustard  plasters  to  the  feet,  cold  applications  to  the  forehead  and  neck ; 
when  necessary,  also,  in  bleeding  (best  by  puncture  of  a  vein).  In  syncope  and 
concussion  of  the  brain,  the  patient  is  placed  in  a  horizontal  position,  is  al- 
lowed to  inhale  ammonia,  the  face  is  sprinkled  with  cold  water,  the  temples 
rubbed  with  alcohol,  the  respiratory  muscles  are  faradized,  and,  when  necessary, 
the  heart's  action  is  stimulated  by  injections  of  ether  and  camphor. 


LECTURE   XX 
Diseases  of  the  Cerebellum 

Gentlemen:  The  progress  which  brain  surgery  has  made  during  recent 
years  has  rebounded  to  the  advantage  of  the  cerebellum  in  special  degree.  This, 
however,  places  not  only  upon  the  neurologist,  but  also  upon  the  general  physi- 
cian, the  duty  of  being  alive  to  the  earliest  possible  recognition  of  cerebellar  af- 
fections, since  the  result  of  an  operative  procedure  depends  chiefly  upon  the  time 
at  which  we  entrust  the  patient  to  the  hands  of  the  surgeon.  On  the  other  hand, 
it  should  not  be  overlooked,  that  every  operation  in  the  posterior  fossa  of  the 
skull  is  accompanied  by  dangers  to  which  no  patient  should  be  lightly  exposed. 
On  this  account  the  differential  diagnosis  of  cerebellar  diseases  is  of  great  prac- 
tical importance. 

From  a  clinical  standpoint  we  are  justified  in  including  certain  extra- 
cerebellar  affections  of  the  posterior  fossa  of  the  skull  under  "cerebellar  affec- 
tions," since  they  manifest  themselves  preponderatingly  through  cerebellar 
symptoms,  I  mean:  1.  Tumors  of  the  cerebello-pontine  angle.  2.  Serous  menin- 
gitis in  the  posterior  fossa  of  the  skull.  Both  disease  conditions,  as  well  as 
abscesses  and  tumors  of  intra-cerebellar  location,  come  within  the  province  of 
surgery,  while  softenings,  hemorrhages,  atrophies  and  ageneses  of  the  cere- 
bellum, as  well  as  cerebellar  encephalitides  and  cerebellar  syndromes  in  malaria, 
are  to  be  cared  for  by  the  physician. 

We  will  take  uj:>  these  disease  processes  in  the  order  of  their  practical  im- 
portance. 

A.     Tumors 

The  cerebellum  is,  during  childhood  and  youth,  the  location  of  predi- 
lection for  tumor  formation  in  the  nervous  system.  Along  with  true  neo- 
plasms— gliomata,  sarcomata,  fibromata — cystic  tumors  occur,  and  as  an 
infectious  granuloma,  solitary  tubercle  is  to  be  considered.  Besides,  there  mu->t 
be  mentioned  as  a  rarity,  a  parasitic  tumor,  echinococcus  of  the  cerebellum. 

Symptomatologically,  the  brain  pressure  syndrome  which  we  have  already 
described  under  tumors  of  the  cerebrum,  makes  itself  first  apparent ;  still,  with 
this,  some  important  criteria  for  the  localization  of  the  pathological  process, 
whether  it  is  in  the  cerebellum  or  in  its  immediate  neighborhood,  usually  must 
be  considered.  So,  headache  in  cerebellar  affections  is  characterized  by  ex- 
traordinary violence  and  persistence  probably  on  account  of  the  richness  of  the 
tentorium  in  sensory  fibers.  Further,  this  pain  presents  its  maximum  severity 
in  the  occipital  region  and  the  back  of  the  neck,  from  whence  it  radiates  some- 
times into  the  upper  part  of  the  back.     Many  times,  also,  the  patients  complain 

306 


DISEASES    OF    THE    CEREBELLUM  307 

of  frontal  headache;  still,  this  is  diffuse,  while  the  occipito-nucheal  pain  usually 
predominates  on  the  affected  side.  It  can  be  accompanied  by  more  or  less 
marked  stiffness  of  the  neck.  Very  frequently  the  back  of  the  head  is  sensitive 
to  percussion  and  pressure ;  still,  I  place  particular  value  upon  finding  the 
characteristic  pain  which  a  pressure  upward  upon  the  mastoid  process  of  the 
affected  side  produces.  Papilloedema  appears  particularly  quickly  and  mark- 
edly in  cerebellar  tumors,  and  even  when  they  are  unilateral  it  is  generally 
bilateral.  Finally,  a  "spinal  fluid  phenomenon"  has  been  described  in  cere- 
bellar tumors  ;  this  consists  in  the  excessively  rapid  fall  of  pressure  after  a 
lumbar  puncture  and  the  abrupt  cessation  of  the  flow  of  the  cerebrospinal  fluid. 
Evidently  the  tumor  presses  the  medulla  suddenly  into  the  foramen  magnum 
and  in  this  way  interrupts  the  communication  between  the  intracranial  and  the 
spinal  fluid.  In  spite  of  all  the  assertedly  infallible  precautions  which  a  few 
specialists  on  lumbar  puncture  have  recommended,  we  had  better  avoid  this 
dangerous  diagnostic  method  just  as  soon  as  we  have  become  convinced  that 
there  is  a  suspicion  of  tumor  of  the  cerebellum,  since  some  cases  of  sudden 
death  have  occurred  under  its  application. 

Among  the  local  symptoms  of  cerebellar  tumors,  cerebellar  ataxia  stands 
in  the  foreground,  as  it  presents  the  cerebellar  symptom  par  excellence.  By 
this  I  mean  only,  that  without  ataxia  no  cerebellar  affection  can  be  diagnosed 
with  certainty;  in  no  way.  however,  that  typical  cerebellar  ataxia  is  foreign  to 
extra-cerebellar  lesions.  The  interruption  of  one  system  of  cerebcllifugal  or 
cerebellipetal  tracts  suffices  to  deflect  the  regulating  activity  of  the  cerebellum, 
whether  this  lesion  has  its  location  in  the  midbrain,  in  the  pons,  in  the  bulb, 
or  even  in  the  spinal  cord.  It  is  all  the  same  to  a  reflex  apparatus,  whether  the 
interruption  has  destroyed  the  reflex  center,  the  afferent  or  the  efferent  fiber 
complex.  We  have  already,  under  the  description  of  Friedreich's  disease  in 
Lecture  VIII,  mentioned  the  clinical  peculiarities  of  cerebellar  ataxia  and  its 
physio-pathological  basis  (page   12Ji). 

While  incoordination  in  consequence  of  a  total  lesion  of  the  posterior  roots 
of  the  spinal  cord  affects  movements  in  their  entirety,  cerebellar  ataxia — par- 
ticularly on  the  trunk  and  in  the  lower  extremities — shows  a  decided  pi'edilec- 
tion  for  the  composite  movements,  that  is.  for  those  movements  which  require 
the  cooperation  of  extended  groups  of  muscles.  In  such  patients  it  is  observed 
that  the  simple  muscular  movements,  for  example,  flexing  or  extending  the 
foot,  the  knee,  the  hip,  adduction  or  abduction  of  the  thigh,  can  be  executed 
correctly;  that,  however,  their  dynamic  and  static  combination  is  disturbed. 
s.i  .He  produced  a  zigzag  gait,  reeling,  more  or  less  marked  swaying,  as  a  con- 
sequence of  an  interruption  of  flic  synergies  which  are  necessary  for  the  im- 
mobilization and  steadying  of  the  body  and  its  members  in  walking  and  al  rest. 
Here,  also,  must  be  considered  that  phenomenon  to  which  attention  has  been 
called  by  Babinski  under  the  name  "cerebellar  asynergy."  Winn  the  patient 
attempts  to  raise  himself  while  [ying*on  his  hack,  he  elevates  his  lower,  extremi 
ties  instead  of  his  trunk;  when  going  forward  he  allows  his  trunk  to  lag  behind 
to  a  certain  extent,  and  so  is  in  danger  of  falling  backward. 

As  a  rule,  cerebellar  ataxia  affects  the  upper  extremities  to  a  mucn  less 
Hegree;  sometimes,  indeed,  it  appears  to  avoid  them  entirely.     It  must  be  con- 


308  LECTURE    XX 

sidered,  however,  that  in  man  the  arms  are  subjected  to  the  coordinating  in- 
fluence of  the  cerebellum  to  a  very  limited  extent  on  account  of  their  little 
importance  in  the  maintenance  of  the  equilibrium.  Nevertheless,  I  have  nearly 
always  been  able  to  detect  certain  atactic  disturbances  in  the  movements  of 
grasping  with  the  hand.  As  to  this,  we  owe  to  Bablnskl  an  ingenious  method 
of  bringing  out  latent  disturbances  of  coordination  in  the  arms.  The  patient 
is  ordered  to  carry  out  in  rapid  succession  muscular  actions  opposed  to  one 
another,  for  example,  pronation  and  supination,  and  it  is  often  noticed  then 
that  the  individual  with  cerebellar  disease  can  no  longer  effect  so  subtile  co- 
operation of  the  antagonistic  muscles.  This  phenomenon  is  called  "adia- 
dochokinesis"    (a=privative ;  dtaoi^rj  ^succession). 

In  clinical  importance  cerebellar  hypotonia  is  inferior  to  cerebellar  ataxia, 
since  the  first  may  be  entirely  obscured  by  simultaneous  lesion  of  the  pyramidal 
tracts;  such  a  lesion,  however,  is  one  of  the  most  frequent  indirect  effects  of 
tumors  of  the  cerebellum,  while,  on  the  other  hand,  marked  spastic  conditions 
are  in  no  way  uncombinable  with  typical  cerebellar  ataxia.  Where,  however, 
cerebellar  hypotonia  is  present,  it  manifests  itself  like  spinal  hypotonia  (see 
above,  page  173)  as  well  in  a  relaxed  condition  of  the  muscles  as  in  the  possi- 
bility of  bringing  the  extremities  into  very  abnormal  positions  on  account  of 
the  relaxation  of  the  antagonists,  for  example,  overextension  or  overflexion. 

A  method  of  examination  recommended  by  Stuart  and  Holmes,  and  of 
which  I  regularly  make  use,  deserves  to  be  mentioned.  If  a  movement  of  flexion 
which  the  patient  is  attempting  to  carry  out  is  opposed  and  the  resistance  is 
suddenly  withdrawn,  the  flexion  takes  place  in  an  extreme  degree  and  the  re- 
action, which  normally  always  occurs,  is  either  absent  entirely  or  there  is  only 
a  trace  of  it.  This  is  a  symptom  which  is  capable  of  demonstrating  to  us  the 
hypotonia  of  the  extensor  muscles. 

I  would  lay  particular  emphasis  upon  the  different  conditions  of  the  re- 
flexes in  spinal  and  in  cerebellar  hypotonia.  The  first  is  accompanied  by 
areflexia  or  hyporeflcxia,  as  we  saw  in  Lecture  XI,  the  last  is  entirely  inde- 
pendent of  the  intensity  of  the  tendon  phenomena  which  can  be  found  not  rarely 
retained  or  even  exaggerated.  Where,  however,  in  cerebellar  tumors,  a  reduc- 
tion or  suspension  of  the  reflexes  is  found,  it  appears  to  be  dependent  upon 
mechanical  indirect  action.  Indeed,  the  increased  brain  pressure  by  extension 
to  the  closed  sac  which  the  spinal  dura  mater  forms  about  the  posterior  spinal 
nerve  roots  may  produce  radicular  lesions  which  can  lead  to  symptoms  of  their 
anatomical  destruction  (Hoche,   Wollcnberg  and  others). 

In  general,  ataxia  and  hypotonia  are  most  marked  in  tumors  of  the  vermis. 
In  tumors  of  the  cerebellar  hemispheres,  they,  as  a  rule,  ai-e  present  in  less 
intensity,  and  indeed,  when  the  process  is  unilateral,  in  the  form  of  hemiataxia 
and  hemihypotonia  of  the  same  side.  According  to  my  personal  experience, 
strict  unilaterality  of  these  symptoms  is  much  rarer  than  their  preponderance 
on  one  side.  This  last  is  the  rule  in  all  those  cases  in  which  the  neoplasm  in- 
deed affects  one  hemisphere  to  a  greater  degree,  but  oversteps  the  middle  line 
to  a  greater  or  less  extent ;  in  tin's  class  are  probably  the  majority  of  cerebellar 
tumors — if  we  leave  out  of  .consideration  those  of  the  cerebello-pontine  angle. 
As  a  matter  of  course,  the  recognition  of  atactic   or  hypotonic  disturbances 


DISEASES    OF    THE    CEREBELLUM  309 

on  tlie  relatively  avoided  side  requires  particularly  careful  methods  of  inves- 
tigation. 

Paradoxical  are  certain  observations  of  tumors  of  the  vermis  without  ataxia 
or  hypotonia.  It  has  been  attempted  to  explain  these  rare  cases  in  different 
ways.  For  example,  Xotlinagel  believed  that  here  the  tumors  were  of  such 
slow  growth  that  a  compensatory  function  could  be  established  by  an  entering 
into  action  of  other  brain  parts.  In  the  case  of  solitary  tubercle  the  absence 
of  atactic  disturbances  has  been  attributed  to  the  intactness  of  the  axis  cylin- 
ders  which  pass  through  the  focus.  Though  these  attempts  at  explanation  may 
he  satisfactory  for  a  certain  number  of  cases  of  tumors  of  the  vermis  which 
have  run  their  course  without  ataxia  and  hypotonia,  they  are  not  suitable  for 
other  cases  which,  on  the  contrary,  were  characterized  bv  an  extremely  rapid 
growth  and  plainly  destructive  nature  of  the  process.  If  later  pathologico- 
anatomical  investigations  do  not  solve  the  riddle,  we  must  satisfy  ourselves  with 
the  hypothesis  that  these  exceptional  cases  affected  individuals  in  whom  the 
cerebellum,  "ab  ovo"  possesses  a  particularly  small  functional  importance. 

Vertiginous  phenomena  are  exceedingly  characteristic  for  cerebellar  tumors, 
even  in  the  initial  stages,  and  indeed  they  are  manifested  as  true  rotary  vertigo, 
"systematic  vertigo,"  produced  by  irritation  of  the  neurones  from  the  nuclei 
of  the  vestibular  nerve,  which  pass  through  the  cerebellum.  The  patients  have 
the  distinct  sensation  of  rotation  both  of  their  own  bodies  and  of  the  surround- 
ing objects  in  a  definite  direction.  These  sensations  set  up  nausea,  often  ac- 
companied by  pallor,  sweating,  vomiting,  etc.  They  depend  upon  the  fact 
that  by  the  irritation  of  the  tumor  the  stimulation  of  the  vestibulo-cerebellar 
apparatus  (which  is  normally  produced  only  by  the  hydrostatic  relations  in 
the  semicircular  canals  of  the  labyrinth)  is  in  contradiction  to  the  actual 
position  of  the  body  and  so  falsifies  the  ocular,  articular  and  muscular  im- 
pressions. Particularly  interesting  an'  the  attacks  of  vertigo  which,  under  the 
names  of  "cerebellar"  or  "vestibular"  attacks,  "cerebellar  fits,"  "cerebello- 
pontile  seizures"  have  been  described  by  Ziehen,  Dana,  limit,  and  others.  The 
Midden  and  extremely  violent  vertigo  which  characterizes  them  is  often  ac- 
companied by  loss  of  consciousness;  as  a  rule,  however,  by  definite  atactic 
disturbances,  severe  vomiting,  headache,  tinnitus  aurium  and  nystagmus. 

This  last  symptom  is  particularly  frequent  in  cerebellar  tumors  and  can 
in  general  he  considered  as  a  symptom  of  irritation  of  "Deiters'  nucleus"  or 
of  the  "posterior  longitudinal  fasciculus."  The  one,  indeed,  lies  close  to  the 
Cerebellum,  the  other,  however,  passes  directly  under  the  vermis  in  the  most 
dorsal   portion  of  the  tegmentum,  to  the  eye  muscle  nuclei. 

Via  the  nucleus  of  Deiters  and  the  posterior  longitudinal  fasciculus,  also, 
in  the  artificial  provoking  of  a  nystagmus  by  the  so-called  Barony's  lest,  the 
impulse  is  transmitted  to  the  ahducens  and  OClllo-motoriuS.  In  healthy  peo- 
ple, by  Syringing  the  external  auditory  canal  with  cold  water,  a  nystagmus 
toward  tin  opposite  side  is  produced;  with  hot  water  there  is  one  toward 
the  side  of  the   injection.      In   tumors  of  the  cerehellum,  however    this  "'caloric 

nystagmus"  may  lie  absent. 

A  spontaneous  nystagmus  of  patients  with  cerebellar  tumors  can  lie  hut 
rarely  noticed  when  looking  to  the   front  and   must   lie  provoked  by  having  the 


310  LECTURE    XX 

patient  look  far  to  the  right  or  to  the  left.  On  this,  a  decided  oscillation  of 
the  eyes  is  observed  when  the  patient  looks  in  the  direction  of  his  cerebellar 
lesion.  I  lay  great  stress  upon  this  point.  On  the  other  hand,  I  have  never 
been  able  to  confirm  another  phenomenon  mentioned  by  different  authors, 
namely,  the  greater  extent  of  the  excursions  in  the  eye  corresponding  to  the 
diseased  half  of  the  cerebellum.  As  Oppenheim  has  emphasized,  a  nystagmus 
which  is  absent  in  a  standing  position  can  sometimes  be  elicited  by  having  the 
patient  lie  upon  his  side. 

Upon  further  growth  of  the  cerebellar  tumor  a  number  of  indirect  symp- 
toms usually  manifest  themselves  in  variable  combination.  Among  the  most 
frequent  is  abducens  paralysis  or,  rather,  a  paralysis  in  looking  toward  the 
cerebellar  focus.  Of  course  the  last  can  give  rise  to  "conjugate  deviation" 
toward  the  opposite  side.  Trochlear  paralysis  occurs  rather  regularly  in 
neoplasms  of  the  anterior  end  of  the  vermis,  besides  this,  also,  sometimes  a 
paralysis  of  the  superior,  inferior,  and  internal  recti  muscles,  on  the  other 
hand,  almost  never  one  of  the  internal  eye  muscles.  Defect  and  irritative 
symptoms  on  the  part  of  the  cranial  nerves  from  the  5th  to  the  12th  develop 
more  rapidly  when  the  neoplasms  are  situated  outside  of  the  cerebellum  than 
when  they  are  intracerebellar ;  they  are,  however,  quite  frequent  here  also. 
Disturbances  of  speech,  swallowing,  respiration  and  articulation  can  produce 
in  such  cases  the  picture  of  bulbar  paralysis  already  considered  in  Lecture  VII. 
Possibly  from  dysarthria  produced  in  this  manner,  the  scanning  speech  which 
has  been  described  by  Dreschfeld  and  Bruns  in  cerebellar  tumors,  and  which 
perhaps  represents  nothing  more  than  a  cerebellar  ataxia  of  the  speech  muscles, 
must  be  distinguished.  The  investigations  of  Hothmann  and  Katzenstein,  who 
have  found  in  animals  a  region  in  the  cerebellum  whose  extirpation  produces 
an  ataxia  of  the  vocal  cords,  indicate  this. 

Frequently  in  cerebellar  tumors  sudden  death  from  pressure  upon  the 
medulla  has  been  observed;  not  rarely  also  in  the  course  of  the  affection,  a 
unilateral  paralysis  or  paresis.  According  to  whether  the  pressure  has  oc- 
curred anterior  or  posterior  to  the  pyramidal  decussation,  the  hemiplegia  or 
liemiparesis  affects  the  limbs  of  the  same  side  or  those  of  the  opposite  side. 

Certain  forced  attitudes  and  compulsory  movements  are  particularly 
marked  when  the  neoplasm  acts  upon  the  anterior  or  the  middle  cerebellar 
peduncle.  They  are,  rolling  about  the  long  axis  or  bending  the  neck  and  trunk 
toward  a  definite  side,  as,  for  example,  in  the  observations  of  Russell  and  Bruns. 
Nevertheless  it  is  impossible  to  give  generally  applicable  rules  with  regard  to 
the  direction  in  which  these  involuntary  attitudes  and  movements  take  place, 
whether  toward  the  healthy  or  toward  the  diseased  side,  and  its  physio-patho- 
logical substratum  is  still  quite  obscure  and  the  subject  of  controversy.  No 
more  definite  is  our  knowledge  of  the  pathogenesis  of  the  choreic-athetoid 
movements  which  according  to  Bonhuffcr,  Pincles,  and  others,  may  occur  on 
the  same  side  upon  destruction  of  one  brachium  conjunctivum.  Also  the  con- 
ditions to  which  the  vertical  divergence  of  the  eyeballs  (of  Magendie)  owes 
its  origin,  are  unknown  to  us.  This  symptom  is  supposed  to  be  found 
along  with  conjugate  deviation  in  affections  of  the  middle  peduncle  of  the 
cerebellum. 


DISEASES    OF    THE    CEREBELLUM  311 

A  definite  category  of  tumors  of  the  posterior  fossa  of  the  skull  manifests 
itself  by  a  rather  stereotyped  clinical  picture,  namely : 

TUMORS  OF  THE  CEREBELLO-PONTIXE  ANGLE 

These  are  neoplasms  (usually  fibroma  or  fibrosarcoma)  which  take  their 
origin  from  the  connective-tissue  sheaths  of  the  auditory  or  the  facial  close  to 
the  exit  of  these  nerves  and  present  the  following  symptoms:  Paresis  in  look- 
ing in  one  direction,  nystagmus,  absence  of  the  corneal  reflex,  disturbances  of 
sensibility  in  the  distribution  of  the  trigeminus,  nervous  hardness  of  hearing 
or  deafness,  adiadochinesis,  all  upon  the  side  of  the  disease  focus,  and  besides 
this,  papilloedema,  cerebellar  ataxia  and  occipital  headache.  Of  course  the 
symptomatology  of  these  tumors  is  not  always  such  a  complete  one ;  on  the 
other  hand,  however,  further  phenomena  ma}'  be  added  to  it,  for  example,  con- 
vulsions, neuralgiform  pains  in  the  trigeminus  region,  anosmia,  etc. 

Differential  Diagnosis 

Confusion  of  cerebellar  tumors  with  multiple  sclerosis  has  frequently  oc- 
curred. We  know,  indeed,  that  this  last  has  in  common  with  cerebellar  neo- 
plasms, the  nystagmus,  cerebellar  ataxia,  vertigo  occurring  sometimes  in  at- 
tacks, that  further,  scanning  speech  also  occurs  in  cerebellar  tumors,  and  that 
their  pressure  upon  the  motor  tracts  of  the  brain  axis  may  produce  increase 
of  reflexes  and  spasticity.  We  might  add  that  upon  a  few  occasions  an  in- 
tention tremor  has  been  observed  in  cerebellar  neoplasms  (whether  as  a  symp- 
tom of  irritation  of  the  pyramidal  fibers  or  not?).  Very  important  for  dif- 
ferential diagnosis  is  examination  of  the  eye  grounds;  though  there  is  ex- 
ceptionally a  swelling  of  the  papilla  in  multiple  sclerosis  also  (probably  from 
complicating  hydrocephalus).  The  headache  in  multiple  sclerosis  is  scarcely 
ever  as  severe  as  in  cerebellar  tumors. 

Particularly  difficult  is  sometimes  the  differentiation  of  cerebellar  tumor 
from  cerebellar  abscess  and  serous  meningitis  of  the  posterior  fossa,  in  case 
Hi''  latter  affections  do  not  come  on  acutely  with  fever  after  an  otitis  or  a 
in  nl  trauma.  The  absence  of  papilloedema  in  doubtful  case-,  speaks  always 
for  abscess,  while  in  serous  meningitis  this  symptom  occurs  to  almost  the  same 
extent  as  in  cerebellar  tumor.  In  favor  of  the  diagnosis,  serous  meningitis,  an 
Outspoken  remitting  then  exacerbating  course  may  be  in  any  event  decisive. 
In  the  great  majority  of  cases  indeed,  it  is  unfortunately  impossible  with 
tumor  symptoms  to  exclude  a  '•pseudo-tumor  meningiticus."  This,  however, 
matters  little,  since  therapeutically  after  failure  of  medical  treatment  directed 
against  serous  meningitis,  an  operation  for  this  is  indicated,  and  its  finding 
after  opening  the  skull  may  prove  a   pleasing  surprise  for  us. 

We  might  mention  the  also  difficult  differentiation  from  aneurisms  of  the 
basal  arteries.  Characteristic  of  the  last  is  a  vascular  murmur  within  the 
skull  synchronous  with  the  pulse.  A  suspicion  in  this  direction  might  be  justi 
tied  when  there  is  advanced  arteriosclerosis  and  increased  blond  pressure,  also 
in  alcoholism  or  syphilis,  finally,  after  injuries  to  the  skull  (fractures  of  the 
base). 


312  LECTURE    XX 

Treatment 

We  will  proceed  here  according  to  the  principles  laid  down  already  when 
speaking  of  tumors  of  the  cerebrum,  that  is.  at  first  we  will  try  the  resorption- 
favoring  action  of  mercury  and  iodide  against  the  scarcely  ever  to  be  ex- 
cluded eventuality  of  serous  meningitis.  But  not  for  too  long  a  time.  Where, 
after  3  or  4  weeks,  definite  reduction  of  the  objective  and  subjective  symptoms 
is  not  apparent,  the  continuance  of  internal  treatment  should  not  be  persisted 
in,  hut  trephining  into  the  posterior  fossa  of  the  skull  should  be  advised. 

Even  if  the  tumor  then  proves  to  be  irremovable,  nevertheless  a  long  period 
of  improvement  may  be  the  result  of  the  decompression,  in  any  case  we  can 
hope  to  relieve  the  patient  of  his  unbearable  headache  and  to  prevent  threat- 
ened blindness.  Now  there  is  in  any  event  danger  connected  with  simple  de- 
compressive craniectomy;  so,  a  boy  with  glio-sarcoma  of  the  vermis  died  sud- 
denly in  collapse,  24  hours  after  making  an  opening  in  the  occipital  bone, 
although  he  apparently  recovered  well  from  the  operation,  and  the  section 
showed,  besides  the  tumor,  marked  hyperemia  of  the  choroid  plexuses  with 
great  hydrops  ventriculorum — perhaps  to  be  considered  as  a  reaction  to  the 
operative  removal  of  pressure.  Many  neurologists  and  surgeons  have  reported 
similar  experiences  and  many  on  this  account  prefer,  even  in  cerebellar  tumors, 
to  make  the  decompression  in  the  temporal  region  of  the  skull ;  indeed,  I  have 
seen  also  in  a  case  of  sarcoma  of  the  left  cerebellar  hemisphere  subjected  to 
temporal  craniectomy,  after  a  few  days  exitus  from  vagus  paralysis  in  conse- 
quence of  hydrops  of  the  4th  ventricle.  Naturally  it  may  be  objected  to 
temporal  craniectomy,  that  in  it  we  either  in  advance  renounce  a  curative 
operation  in  favor  of  a  palliative  one,  or  postpone  the  first  by  addition  of  the 
last  and  subject  the  jDatient  to  two  operations  instead  of  one.  In  so  unfavor- 
able a  disease,  in  any  case  some  courage  is  needed.  Now  as  to  the  curative 
effects  of  the  radical  procedure,  they  are,  as  already  expressly  emphasized, 
dependent  upon  the  time  of  the  intervention,  and  many  bad  results  would 
perhaps  be  avoided  by  early  diagnosis.  The  pathologico-anatomical  nature 
of  the  tumor  is  also  important ;  cysts  and  fibroma  give  the  best  prognosis ; 
after  them,  solitary  tubercle  (which  can  spontaneously  cease  growing),  gli- 
omata  which  pass  over  into  normal  tissue  without  sharp  limitation,  the  worst; 
again,  the  location  of  the  tumor  plays  a  great  role — so  the  operation  for  cere- 
bellopontine tumors  in  spite  of  their  usually  good  limitation  and  often  easy 
separation  is,  on  account  of  the  neighborhood  of  the  medulla  and  the  difficulty 
of  access  to  the  tumor,  always  a  severe  procedure,  which  is  usually  followed 
by  death  from  heart  failure.  This  happened  in  two  cases  observed  by  me, 
though  the  operation  was  undertaken  with  local  anesthesia,  and  F.  Krause  in 
50  operative  cases,  has  lost  46  (that  is,  92  per  cent.).  The  operation  in  two 
stages  is  preferred  by  most  surgeons  upon  intervention  in  the  posterior  fossa  of 
the  skull. 

B.      Abscesses  of  the  Cerebellum 

They  occur  like  cerebral  abscesses,  either  by  metastasis  (particularly  after 
lung  abscess   and  gangrene)    or   after  injuries   to   the   skull,  which  sometimes. 


DISEASES    OF    THE    CEREBELLUM  313 

have  occurred  years  or  decades  before.  Most  frequently,  however,  cerebellar 
abscess  is  the  result  of  purulent  middle  ear  inflammation  extending  through 
the  petrous  bone  and  so  presents  the  counterpart  of  otitic  abscess  of  the 
temporal  lobe.  The  development  can  be  very  acute,  or  the  symptoms  may 
arise  gradually  in  the  course  of  several  weeks;  finally  there  are  very  gradual 
forms  which  stretch  over  years.  Through  encapsulation  such  abscesses  may 
indeed  become  latent ;  in  these,  however,  sudden  lighting  up  of  the  acute  process 
which  then  usually  leads  to  perforation  of  the  abscess  wall  and  to  death  from 
purulent  meningitis,  is  frequent.  The  symptoms  are  headache,  stiff  neck,  ver- 
tigo, vomiting,  dysarthria,  disturbances  in  swallowing,  paralysis  in  looking 
toward  the  affected  side  (important  for  localization)  ataxia.  Rarer  are 
nystagmus,  hemiataxia  upon  the  side  of  the  abscess,  papilledema  and  loss  of 
the  patellar  reflexes.  Fever  is  important  diagnostically,  but  no  regular  phe- 
nomenon; indeed,  according  to  Macewen,  subnormal  temperatures  occur. 
Against  brain  puncture  which  naturally  is  capable  of  confirming  the  diag- 
nosis in  the  first  place,  Fcdor  Krause  wains  on  account  of  the  danger  of  an 
infection  of  the  meninges.  Operation  is,  of  course,  strictly  indicated,  but  un- 
fortunately offers  only  slight  probability  of  cure. 

C.      Serous  Meningitis  of  the  Posterior  Fossa  of  the  Skull 

The  sacculations  of  the  pia  arachnoid  in  the  neighborhood  of  the  cere- 
bellum— first,  the  "cisterna  acustico-facialis"  at  the  cerebello-pontine  angle, 
second,  the  "cisterna  cerebello-medullaris"  between  the  medulla  and  the  cere- 
bellum—  present  a  peculiar  seat  of  predilection  for  retention  cysts.  These  last 
are  of  inflammatory  nature,  depend  upon  the  closing  off  of  these  preformed 
arachnoidal  spaces  along  with  corresponding  inhibition  of  the  resorptive  pro- 
Cesses  and  are  caused  by  traumatic,  otitic  and  syphilitic  infection.  Fig.  87 
shows  a  case  of  such  cystic  serous  meningitis  formerly  published  by  me.  In  an 
ideal  sagittal  cross-section  in  which  trephining,  with  separation  of  the  fiat 
adhesions  which  stretched  over  from  the  region  of  the  pyramis  and  the  lobuli 
bivc  litres  cerebelli  to  the  posterior  limit  of  the  medulla,  had  as  a  result  dis- 
appearance of  all  the  symptoms  pointing  to  a  space-narrowing  process  in  the 
posterior  fossa  of  the  skull.  When,  six  months  later,  attacks  of  vertigo  and 
headache.  (I  vsdiadochokincsis,  and  slight  cerebellar  gait  occurred  again,  an 
iodide  cure  caused  rapid  disappearance  of  these  symptoms.  There  was  plainly 
onlv  a  slight  accumulation  of  exudate  within  partial  adhesions  in  the  operative 
li(  Id.  Slight  cases  of  serous  meningitis  can  also,  from  the  beginning,  he  brought 
to  recovery  by  purely  interna]  treatment  (in  which,  even  in  nonsyphilitic  cases, 
along  with  iodide  of  potassium,  mercury  certainly  works  favorably);  however, 
such  treatment  if  it  gives  no  result  should  not  he  loo  long  continued  (especially 
when  there  is  definite  papilledema).  Also,  ;i  decided  tendency  to  relapse  may 
justify  a  decision  to  operate  even  in  such  cases  as  promptly  read  to  rcsorhcnl  s, 
111    the    more,    since    the    results    of    these    operations    ;ire    very    good.       This    is 

shown  not  only  by  mv  ease,  hut  also  by  those  of  Placzek-Krause,  Opperiheim, 

lion  liimll.  and  others.     Svmpt atologically  a  differentiation  from  tumor  of 

the  cerebellum  or  of  the  cerebello-pontine  angle  is  usually  not   possible.     The 


314 


LECTURE    XX 


diagnosis  can  nevertheless  be  made  from  the  anamnesis,  the  remittent-exacer- 
bating course,  also  ex  juvantibus.  In  my  case,  besides  this,  the  relatively 
slight  degree  of  cerebellar  ataxia  after  one  year's  duration  of  the  disease, 
the  absence  of  spontaneous  nystagmus,  the  exceedingly  marked  facial  paresis ; 
further,  the  comparatively  equal  distribution  of  the  symptoms  upon  the  right 
and  upon  the  left,  had  so  impressed  upon  me  the  idea  of  an  extracerebellar  and 
median  location  of  the  expansive  process  that  I  could  make  a  probable  diag- 
nosis of  serous  meningitis  and  hydrops  of  the  4th  ventricle.      This  last  con- 


_Sin  occ. 


Cyste 


Serous  Meningitis  of  the  Posterior  Fossa  of  the  Skull.    Ideal  Sagittal  Section. 
Kh.  =  Cerebellum.     Cyste  =  Cyst. 

dition,  as  Fig.  87  shows,  was  in  fact  present  and  is  a  usual  accompaniment  of 
cysts  in  the  region  of  the  cisterna  cerebello-medullaris. 


D.      Hemorrhages  and  Softenings 

Hemorrhages  into  the  cerebellum  occur  under  the  same  conditions  as  those 
into  the  cerebrum  (miliary  aneurisms  of  the  artery  of  the  dentate  nucleus  play 
of hn  an  important  role),  but  are,  however,  much  rarer.  The  lamellar  structure 
of  the  cerebellum  brings  it  about  that  the  blood  breaks  through  outward  or  into 
the  4th  ventricle.  Softenings  in  the  cerebellum  are  even  rarer.  This  depends 
on  the  one  hand  upon  the  fact  that  the  cerebellar  arteries  are  given  off  from 
the  basilar  artery  at  an  angle  which  docs  not  favor  the  entrance  into  them  of 
embolisms ;  on  the  other,  there  are  numerous  anastomoses  between  the  individual 
arteries  of  the  cerebellum.  Slight  hemorrhages  and  softenings  in  the  cere- 
bellum may  manifest  themselves  clinically  only  by  a  short  period  of  uncon- 
sciousness or  an  attack  of  vertigo  and  leave  behind  no  residua,  so  that  such 
lesions  occasionally  are  noticed  only  as  accidental  autopsy  findings.  As  pro- 
dromal symptoms  of  cerebellar  hemorrhage,  Mingazzini  has  mentioned  occipital 
headache  in  two-thirds  of  his  cases  lasting  for  months,  even  for  years.  Remafc 
has  observed  occasionally  repeated  severe  vomiting.  Lussuna  considers  severe 
and  repeated  attacks  of  vertigo  a  sign  of  arteriosclerosis  of  the  cerebellum. 
In  very  severe  cerebellar  hemorrhage,  death  may  occur  suddenly ;  Mingazzini 


DISEASES    OF    THE    CEREBELLUM  315 

describes  a  case  in  which  an  old  woman  suddenly  turned  around  several  times, 
and  then  fell  dead.  In  other  cases  of  cerebellar  hemorrhage,  as  well  as  in 
many  instances  of  cerebellar  softening,  definite  functional  disturbances  follow 
an  ictus,  for  example,  staggering  gait,  conjugate  deviation  of  the  eyes,  nys- 
tagmus, forced  turning  of  the  head  and  neck.  These  phenomena  can  dis- 
appear by  degrees. 

E.     Agenesis  and  Atrophies 

Congenital  cerebellar  defects  may  remain  latent  even  when  they  affect 
an  entire  half  of  the  organ.  If  they  extend,  however,  to  both  halves,  they 
usually  manifest  themselves  by  cerebellar  ataxia.  A  few  cases,  however,  do 
not  show  this  even.  Sclerotic  atrophies  of  the  cerebellum  can  equally,  although 
more  rarely,  remain  latent  clinically,  especially  when  they  occur  at  an  advanced 
age  and  may  be  considered  as  degenerative  processes  without  inflammatory  basis. 
In  general,  however,  they  cause  more  severe  atactic  disturbances  than  the 
congenital  ageneses,  and  even  when  they  are  only  unilateral.  Sometimes,  be- 
sides this,  other  symptoms  are  also  noticed,  for  example,  speech  disturbances 
and  choreiform  movements.  Often  such  patients  have  epileptic  attacks  which 
indicates  involvement  also  of  the  cerebral  cortex — even  when  this  escapes  recog- 
nition by  the  histological  methods  at  our  disposal.  In  any  event,  combination 
of  atrophy  of  the  cerebellum  with  sclerotic  atrophy  in  the  cerebrum  and  spinal 
cord  is  not  at  all  rare.  Here  the  clinical  picture  is  richer  in  symptoms,  in  that 
tremor,  nystagmus,  weakmindedness,  paresis,  etc.,  are  also  present.  To  enter 
into  the  different  disease  pictures  which  it  has  been  attempted  to  isolate  as 
cerebello-spinal,  olivo-ponto-cerebellar,  cerebro-cerebellar  atrophies,  etc.,  does 
not  correspond  to  the  practical  end  held  in  view  in  these  lectures.  It  is  a 
difficult  and  imperfectly  investigated  subject  which  is  connected  at  many  points 
with  the  hereditary  forms  of  ataxia  which  we  have  discussed  in  Lecture  VIII. 

That  even  total  defects  of  the  cerebellum  can  remain  latent,  in  case  the 
rest  of  the  central  nervous  system  presents  no  defect,  is  explainable  from  the 
slight  functional  dignity  of  the  cerebellum.  This  is  taken  strictly,  neither 
motor  nor  sensory  in  function;  neither  is  it  possible,  at  least  in  man,  to  sepa- 
rate limited  centers  of  specific  activity.  In  animals  there  is,  nevertheless,  ac 
cording  to  Hoik,  van  Rynberle,  Rothmann,  Lourie,  and  others,  a  certain  cor- 
tical localization  possible,  which,  however,  with  ascent  in  the  animal  kingdom, 
diminishes  in  definiteness.  The  results  of  the  best  experimenters,  however  much 
tiny  differ  from  one  another  in  point  of  detail,  agree  in  showing  that  the 
cerebellum  is  only  able  to  act  in„a  modifying  manner  upon  the  cerebrospinal 
unction.  Luciani  first  attempted  to  determine  precisely  the  influence  of  the 
Cerebellum  upon  the  rest  of  the  nervous  system  and  distinguished  a  sthenic, 
tonic-  and  static  action.  The  cerebellum,  according  to  fhis  author,  reinforces 
the  potential  energy  of  the  brain-spinal  cord  innervation,  increases  the  neuro 
muscular  tonus,  and  aids  the  continual  blending  of  the  motor  impulses.  The 
elimination  of  the  cerebellum  has  on  this  account  asthenic-,  atonic  and  astasic 
disturbances  as   a    result.      Then   Thomas   has  defended    the  view   that    the   triad 

of  Luciani  depends  only  indirectly  on   the  suspension  of  cerebellar  function. 


316  LECTURE    XX 

He  has  shown  that  the  cerebellum  is  a  reflex  center  in  the  service  of  preserving 
the  equilibrium,  that  it  receives  peripheral  and  central  impulses  and  reacts 
to  both,  that  it  is  not  the  seat  of  a  special  sense,  but  that  of  a  special  reaction, 
that  this  last,  finally,  serves  equilibration  in  the  different  positions,  both  on 
reflex,  automatic  and  voluntary  movements.  The  animal  without  a  cerebellum 
owes,  according  to  Thomas,  to  his  cerebellar  ataxia,  the  weakness,  incomplete- 
ness and  disharmony  of  his  muscular  contractions  also ;  must,  indeed,  to  a 
certain  extent,  try  his  muscles  out  anew.  Later,  Hermann  Murik  has  laid 
stress  upon  the  fact  that  the  ataxia  of  the  animal  without  a  cerebellum,  in 
whom  injury  to  neighboring  structures  has  been  avoided,  is  limited  to  the 
muscles  of  the  vertebral  column  and  the  limbs.  It  affects  the  "composite  move- 
ments" in  the  service  of  maintaining  the  equilibrium  on  standing  and  walking. 
This  finding  agrees  best  with  the  clinical  studies  on  cerebellar  ataxia.  The 
preponderant  importance  of  this  last  in  the  circle  of  all  the  cerebellar  affections, 
even  the  agencses  and  atrophies,  stands  in  agreement  with  the  result  of  my 
experiments  in  cutting  the  spino-cerebellar  tracts  in  animals.  As  these  tracts 
represent  the  afferent  portion  of  the  reflex  for  the  cerebellar  coordination  of 
the  trunk,  so  the  cerebellar  influence  in  maintaining  the  equilibrium  of  the 
head  and  neck  is  regulated  through  the  vestibulo-cerebellar  fibers.  This,  the 
experiments  of  Ewald  and  others  have  made  clear. 

F.      Infectious  Diseases  of  the  Cerebellum 

There  is  a  cerebellar  form  of  acute  infantile  paralysis  into  which  we  have 
already  gone  when  mentioning  the  Hcine-Medin  disease.  An  encephalitis  can 
also  localize  itself  in  the  cerebellum;  its  differentiation  from  abscess  of  this 
region  can  be  very  difficult,  even  almost  impossible,  as  is  sometimes  even  that 
from  cerebellar  serous  meningitis,  although  in  such  encephalitis  a  papilledema 
occasionally  occurs  (Op.penheim  and  others).  Italian  neurologists  (Puii.tini, 
Forli,  etc.)  have  pointed  out,  finally,  an  acute  cerebellar  symptom-complex 
in  consequence  of  malaria,  in  which  after  a  sudden  febrile  beginning  (with 
Plasmodium  in  the  blood)  there  appear  cerebellar  disturbance  of  gait,  muscu- 
lar atony,  nystagmus,  tremor,  dysarthria,  and  swaying  of  the  head.  This  syn- 
drome reacts  well  to  quinine,  speedily  disappears,  but  has  a  great  tendency 
to  recurrence. 


LECTURE  XXI 

Malformations,  Congenital  and  Early  Acquired 
Defective  Conditions 

A.      Hydrocephalus 

In  considering  the  disease  condition  known  as  hydrocephalus  character- 
ized by  an  excessive  collection  of  fluid  within  the  skull,  we  will  in  the  first  place 
confine  ourselves  to  the  congenital  form.  It  is  by  far  the  most  frequent,  but  is 
not  always  separable  from  the  early  acquired  forms. 

The  pathological  anatomist  distinguishes  a  meningeal  or  external  hydro- 
cephalus from  a  ventricular  or  internal  hydrocephalus.  The  first  is  rare, 
reaches  on  an  average  only  moderate  intensity,  and  is  clinically  not  to  be 
differentiated  with  certainty  from  internal  hydrocephalus.  As  a  rule,  external 
hydrocephalus,  also  called  "intra-meningeal  hygroma,"  is  a  consequence  of 
malformations  of  the  brain,  for  example,  microcephalus ;  we  speak  then  of 
a  "hydrocephalus  ex  vacuo."  The  possibilities  for  the  production  of  a  ven- 
tricular hydrocephalus  are  manifold.  So,  it  can  represent  a  standstill  of 
brain  development  at  the  stage  of  membranous  vesicle;  on  the  other  hand, 
however,  it  may  be  the  result  of  a  foetal  or  early  infantile  ependymitis  or  menin- 
gitis (  the  choroid  plexuses  of  the  ventricle  are  morphologically  the  result  of  the 
invagination  of  the  delicate  membranes  of  the  brain).  Further,  it  can  be  a 
hydrops  by  stasia  through  interruption  of  the  venous  discharge  channel  from 
tin  choroid  plexus  and  the  ventricle  walls;  finally,  it  is  the  result  of  the  ob- 
struction of  those  orifices  through  which  the  infra-cerebral  cavities  commu- 
nicate with  the  subarachnoid  space. 

In  the  different  directions  hereditary  syphilis  appeal's  to  play  the  most 
Important  etiological  role;  v.  Barensprv/ng  found,  among  !)!)  hereditary  luetics, 
4  congenita]  cases  of  hydrocephalus;  Eisner,  among  IS  hydrocephalics,  •'{ 
children  with  manifest  signs  of  syphilis;  Dean,  in  It  hydrocephalics,  obtained 
1  times  a  positive  Wassermann  reaction.  In  the  second  place,  parental  alco- 
holism must  he  mentioned.  Rarely  can  trauma  or  acute  infection  which  have 
acted  upon  the  pregnant  mothe>  he  made  responsible.  The  connection  with 
mental  shocks  during  pregnancy  is  scientifically  not  proven.  A  few  authors 
have  called  attention  to  hereditary  family  eases  of  the  disease. 

The  ventricle  walls  can  he  so  thinned  in  internal  hydrocephalus  that  the 
brain  has  become  a  fluctuating  vesicle.  The  amount  of  fluid  varies  between 
50  to  100  (a-  and  10  liters   -the  average,  according  to  Oppenheim,   is  about 

1    liter. 

\"o    wonder    that    the    skull    can    reach    enormous   dimensions.      The    eircuin- 

:il7 


318 


LECTURE    XXI 


ference  of  the  head  of  the  normal  new-born  infant,  taken  about  the  glabella 
and  the  external  occipital  protuberance,  measures  34  cm,  and  in  the  course 
of  the  first  year  reaches  45  cm.  In  hydrocephalus  circumferences  of  60  and 
70  cm  are  not  rare.  A  16-months-old  patient  of  Frank  had,  indeed,  a  measure 
of  154  cm.  Since  only  the  skull  proper  enlarges  while  the  face  remains  small, 
the  head  assumes  a  characteristic  pear-shape;  the  thin  bones  of  the  skullcap 
can  become  parchment-like,  the  whole  cranium  is  translucent  by  transmitted 
light.  The  sutures  and  the  fontanelles  gape  open,  the  skin  of  the  forehead 
and  the  scalp  becomes  thin  and  atrophic,  and  all  the  more  prominent  appear 
the  dilated  veins.  Since  in  young  children  the  neck  muscles  are  not  able 
to   support   the  head  of  adult   size,  it  wobbles   helplessly   about.      Sometimes,, 


Figs.  88  and  89. 
Congenital  Hydrocephalus. 


on  palpation,  fluctuation  is  perceived,  while  on  auscultation  a  vascular  mur- 
mur is  heard. 

The  skull  deformity  exerts  peculiar  morphological  reactions  upon  the  eye 
and  ear.  The  eyeball  is  forced  down  and  forward,  the  upper  lid  can  only 
incompletely  cover  it.  A  large  portion  of  the  sclerotic  is  always  visible  and 
the  upper  part  of  the  cornea  rising  over  the  border  of  the  lower  lid  gives 
a  picture  of  the  "rising  sun."  The  (often  much  deformed)  ears  are  placed 
strikingly  far  back  and  low  on  the  head  (see  Figs.  88  and  89). 

These  great  morphological  anomalies  correspond  to  marked  functional  dis- 
turbances. As  to  the  intellectual  capabilities,  it  is  indeed  a  fact  that  after 
recovered  slight  hydrocephalus,  striking  mental  capacity  has  been  observed 
(which  is  known  to  have  been  the  case  in  Cuvier  and  Hclmlioltz),  but  perma- 
nent more  or  less  gross  defects  are  the  rule  and  feeble-mindedncss,  imbecility 
or  idiocy  is  the  usual  lot  of  hydrocephalics  who  do  not  die  in  early  childhood. 
Of  41  hydrocephalics,  according  to  Wyss,  only  5  could  go  to  school.  Dis- 
turbances of  the  cranial  nerves  are  often  observed,  for  example,  dilatation 
or  contraction  of  the  pupils  or  lack  of  all   reaction,  nystagmus,  strabismus, 


HYDROCEPHALUS  319 

grimacing  (irritative  facial  symptoms?).  In  a  remarkable  manner  hearing  is 
almost  always  retained  and  only  exceedingly  rarely  is  there  change  in  the 
optic  nerve  (papilledema,  atrophy).  Spastic  conditions  of  the  muscles  of 
the  trunk  and  extremities  are  frequent;  they  correspond  to  the  clinical  picture 
which  we  will  describe  in  the  next  lecture  on  infantile  cerebral  paralyses  and 
Little's  disease.  Epileptiform  attacks,  paroxysmal  vomiting  and  the  occur- 
rence of  severe  headaches  should  indicate  sudden  increase  of  brain  pressure. 
Young  children  give  evidence  of  the  last  by  whimpering  and  putting  the  hand 
to  the  head.  Unusual  symptoms  are  disturbances  of  coordination,  tremor, 
paralyses,  and  (apart  from  the  not  infrequent  hyperesthesia  of  the  integument) 
disturbances  of  sensibility.  Frequently,  on  the  contrary,  there  is  incontinence 
of  faces  and  urine,  as  well  as  a  decided  tendency  to  bed-sores ;  the  last  occur 
upon  the  skull  also   (parietal  bosses,  occiput). 

A  word  on  the  hydrocephalic  fluid  which  is  obtained  on  lumbar  or  brain 
puncture.  It  is,  as  a  rule,  colorless  to  a  slight  amber  tinge,  without  clouding, 
only  rarely  it  contains  a  few  flocculi.  The  content  of  albumen  (globulin)  is 
practically  none,  or  very  little  (not  over  1  per  cent.).  The  specific  gravity 
lies  between  1,001  and  1,009.  The  fluid  contains  further  glucose  and  milk 
sugar.  The  cytological  findings  are  limited  to  the  occasional  presence  of  a 
few  leucocytes. 

How  relatively  frequent  hydrocephalus  is  at  birth  is  shown  best  by  the 
statements  of  the  obstetrician  Rwnge,  according  to  which  a  disturbance  in 
d<  livery  in  consequence  of  hydrocephalus  comes  under  observation  about  once 
in  300  deliveries.  In  this  connection  it  should  be  remembered  that  a  great 
many  congenital  hydrocephali  are  at  birth  of  still  too  moderate  dimensions 
to  cause  any  disturbance.  In  any  event,  the  post-natal  growth  of  congenital 
liuli  ocephali  plays  a  great  role:  this  can  amount  to  1  cm  a  week  and  corre- 
sponds to  an  extra-uterine  duration  of  the  original  disease  process.  The 
mortality  of  hydrocephalics  is  a  very  considerable  one.  At  birth  there  may 
be  bursting  of  the  head  if  the  child  is  not  sacrificed  by  the  obstetrician  by 
p<  rforation  and  cranioclasis  to  prevent  rupture  of  the  uterus.  Hydrocephalics 
born  alive  often  die  during  the  first  days,  weeks  or  months;  the  remainder  of 
the  more  severe  eases  are  taken  away  before  the  third  year  of  life;  only 
slight  eases  can  recover;  usually,  however,  it  is  a  "recovery  with  defect." 
Not  to  be  underestimated  is  the  danger  of  relapse,  renewed  progressive  ad- 
vance which  threatens  during  all  of  childhood.  As  immediate  causes  of  death 
should  be  mentioned:  increasing  brain  pressure  and  coma,  bed-sores  and 
hjfection,  laryngismus  stridulus;  status  epilepticus,  attempts  at  operative 
treatment,  particularly,  however,  intercurrent  affections,  as  gastro-enteritis 
and  bronchopneumonia.  In  one  case  there  was  bursting  of  the  head.  Hare 
but  interesting  are  the  so-called  '•spontaneous  recoveries"  in  which  the  hydro- 
cephalic fluid,  by  wearing  away  of  its  enclosing  case,  makes  a  path  out  either 
throu»h  the  nose,  through  the  orbit,  or   through   the  coronal  or  sagittal   suture. 

From  the  point   of  view  of  differentia]  diagnosis,  there  come  into  consid 
(•ration  before  everything  else  the  rachitic  enlargement   of  the  skull,  in  which, 
However,   the  cranium  assumes  the  characteristic  "box    form,"   the  open   fon- 
fcnelles  are  not  bulging,  and  the  hydrocephalic  position  of  the  eyes  is  absent  ; 


320  LECTURE    XXI 

also  the  so-called  "steeple-skull"  (oxycephalies)  should  not  lead  to  confusion 
with  hydrocephalus,  although  it  is  affirmed  that  this  deformity  represents  the 
reaction  of  the  rachitic  bones  of  the  skull  to  a  moderate  hydrocephalic  press- 
ure (Meltzer).  It  is  to  be  emphasized  that  oxycephalus  is  almost  never  ac- 
companied by  idiocy,  but,  on  the  contrary,  very  frequently  by  optic  atrophy. 
We  would  mention  further  the  peculiar  skull-form  of  hereditarily  syphilitic 
children,  which  Fournier,  on  account  of  the  abnormally  high  and  bulging 
forehead,  has  called  the  "Olympic"  forehead  ("front  olympien"),  and  would 
allude  further  to  the  idiots  with  sclerotic  hyperplasia  of  the  brain  and  great 
skull  dimensions  denominated  by  Virchoic  "cephalones,"  and  finally,  the  so- 
called  "cleido-cranial  dysostosis,"  a  heredo-family  anomaly  in  formation  de- 
scribed by  Marie  and  Sainton,  in  which  persistence  of  the  fontanelles  and 
excessive  width  of  the  skull  is  accompanied  by  congenital  defects  of  the 
clavicles. 

The  non-congenital  hydrocephalus  of  childhood  is  most  frequently  of  men- 
ingitic  origin,  which  can  manifest  itself  clinically  by  acute  onset  with  fever, 
stiff  neck  and  convulsions.  After  the  passing  off  of  this  infectious  stage,  dur- 
ing which  the  spinal  fluid  is  usually  characterized  by  richness  in  albumen,  and 
has  also  been  found  to  contain  bacteria  (for  example,  meningococci),  there 
occurs  a  progressive  increase  of  fluid  within  the  skull  which,  by  degrees,  may 
produce  the  same  symptom-complex  as  the  congenital  form.  Only  in  children 
beyond  the  second  year  of  life  the  firm  closure  of  the  sutures  and  fontanelles 
which  has  already  occurred,  causes  a  plainly  different  disease  picture.  Only 
very  exceptionally  are  the  sutures  forced  open ;  as  a  rule,  indeed,  the  skull 
responds  to  increase  of  pressure  in  its  interior  by  abnormally  rapid  growth ; 
nevertheless,  such  monstrous  dimensions  as  those  in  hydrocephalus  of  the 
first  period  of  life  are  not  nearly  reached,  neither  does  the  typical  eye  position 
occur.  All  the  more  intensely,  however,  does  the  increase  of  the  intracranial 
hypertension  make  itself  felt ;  severe  headaches,  tinnitus  aurium,  vertigo,  vom- 
iting, confusion,  optic  atrophy,  spastic  rigidity  of  the  extremities,  name!}', 
the  lower  ones,  epileptiform  attacks,  etc.,  make  the  differential  diagnosis  from 
brain  tumor  uncommonly  difficult.  Here  and  there,  indeed,  the  hydrocephalus 
distinguishes  itself  from  tumor  formation  by  considerable  variation  in  the 
intensity   of  the   disease   manifestations. 

Into  the  treatment  of  hydrocephalus  we  can  enter  with  but  very  faint 
hope.  In  every  case  we  begin  witli  iodide  and  mercury,  which  even  in  non- 
syphilitic  cases  appear  to  exercise  a  favorable  influence.  From  the  start  large 
doses  are  called  for  (0.15  to  0.2  KI  per  diem  for  children  in  the  first  year, 
0.2  to  0.25  in  the  second  year,  ungt.  hydrarg.,  1.0  a  day  by  inunction).  Sub- 
limate baths  (1.0  HgCk  per  bath)  have  but  slight  activity,  internal  mercurial 
treatment  is  not  to  be  recommended  on  account  of  the  tendency  of  such  chil- 
dren to  gastro-enteritis. 

Derivative  measures  whose  activity  cannot  be  entirely  denied  are  penciling 
the  scalp  with  tinct.  iodi,  and  the  exposure  of  the  occipital  region  to  the  sun 
for  15  to  20  minutes  a  day  as  recommended  by  Somma. 

More  rational,  however,  is  repeated  lumbar  puncture  (25  to  50  cc  spinal 
fluid  drawn  off  at  intervals  of  3  weeks),  while  the  incision  of  the  dural  sac 


MALFORMATIONS,  ACQUIRED   DEFECTIVE   CONDITIONS     321 

to  produce  permanent  drainage,  as  recommended  by  Quincke,  does  not  appear 
to  promise  much  result.  Puncture  of  the  ventricles  is  reserved  for  those  cases 
in  which  increasing  symptoms  of  brain  pressure  or  papilledema  appear  to 
call  for  relief,  but  in  which  on  account  of  imperfect  communication  between 
the  ventricles  and  the  spinal  dural  sac  this  cannot  be  accomplished  by  lumbar 
puncture.  Ventricular  puncture  is,  indeed,  a  dangerous  procedure,  which 
frequently  leads  to  fatal  collapse  or  to  status  epilepticus ;  in  any  case  the 
following  precautions  should  be  observed:  the  most  rigid  asepsis,  puncture 
3  to  4  cm  laterally  from  the  large  fontanelle,  that  is,  below  the  longitudinal 
sinus;  slow  withdrawal  of  at  most  100  cc  fluid;  accurate  watching  of  pulse 
and  respiration ;  in  threatening  collapse  a  partial  replacement  of  the  fluid  by 
physiological  salt  solution.  After  the  operation  an  elastic,  yielding  bandage 
should  be  placed  about  the  skull,  but  the  dangerous  compression  by  adhesive 
plaster,  after  Trousseau,  should  be  avoided  (brain  pressure,  bed-sores).  In 
conclusion,  the  methods  of  "permanent  drainage  of  the  ventricle,"  recom- 
mended by  surgeons,  may  be  mentioned;  these  are:  draining  into  the  subdural 
space  (Kocher),  under  the  scalp  (Mikulicz),  into  the  longitudinal  sinus  (Pa/jr), 
and  finally,  the  so-called  "Balkenstich"  (puncture  through  the  corpus  callo- 
sum)   (Anton  and  Bramann). 

Since  in  marked  hydrocephalus  the  existing  defects  in  the  psychical  sphere, 
even  though  the  disease  process  may  cease,  can  only  to  the  slightest  extent  be 
removed  by  any  method  of  treatment,  an  ethically  thinking  physician  will  enter 
into  the  task  of  making  possible  the  further  vegetative  existence  of  a  paralyzed 
and  idiotic  being  only  with  repugnance. 


B.      Cranial  and  Spinal  Ectopies 

Upon  the  basis  of  preformed  defects  of  the  skull  partial  escape  of  its  con- 
tents can  occur  intra-uterine  and  be  present  at  birth.  We  speak  of  a  cranial 
meningocele  when  only  the  brain  membranes 
air  displaced  outward  (in  this  the  dura 
mater  i>  usually  absent),  of  hydrencephalo- 
c  le  when  tin/  hydropic  ventricle  also  pro- 
trudes. By  enccphalocele  is  meant,  on  the 
contrary,  an  ectopy  of  solid  brain  substance 
which  dors  not  include  any  part  of  the  ven- 
tricle. These  malformations  can  he  located 
on  the  most  varied  parts  of  the  skull.  They 
are  most  frequently  found  in  tlit  occipital 
region,  sometimes  above  and  sometimes  be- 
low the  squama  occipitalis  (see  Fig.  90); 
again  liny  appear  between  the  nose  and  I  he 
orbit  or  between  the  nose  and  the  frontal 
hone  Ectopies  into  the  nasal  cavity,  tlir  mouth  and  in  tin  temporal  region 
Ire  rarer.  Cystic  protrusions  of  the  brain  and  brain  membranes  arc  greatly 
mcreased  in  dimensions  after  birth.  Pedunculated  are  the  meningoceles,  which 
occur  particularly  in  the  occipital  region.     This  form  i--  the  mildest,  since  it 


Fin.  no. 
Superior  Occipital   Hydrocephalocele 


322 


LECTURE    XXI 


is  compatible  with  normal  brain  activit}'.  Often  enough,  indeed,  it  is  combined 
with  hydro-  or  microcephaly  which,  along  with  other  developmental  disturb- 
ances, are  almost  regular  accompaniments  of  the  ectopies  containing  brain 
substance.  The  worst  prognosis  is  given  by  the  hydrencephaloceles ;  their 
post-natal  growth  provokes  brain  pressure  symptoms  which  lead  to  death 
within  a  few  months. 

Good  results  can  be  expected  only   from  the  operative  removal  of  simple 
meningoceles ;  these  should  be  early  transferred  to  the  surgeon,  as  otherwise 


Fig.  91. 
Occult  Spina  Bifida  with  Local  Hypertrichosis. 

there  is  a  risk  of  rupture  of  the  cyst  with  unavoidable  meningeal  infection. 
Spontaneous  cures  are  excessive  rarities.  As  clinical  criteria  of  meningoceles 
it  is  noticed  that  they  fluctuate  always  in  contradistinction  to  the  encephalo- 
celes,  and  they  are  distinguished  from  hydrencephaloceles  by  being  usually 
pedunculated  and  often  translucent;  also  they  decrease  in  size  on  pressure, 
and  can  even  be  replaced.  In  the  last  method  of  examination  there  is,  indeed, 
need  for  great  caution  on  account  of  the  danger  of  provoking  brain  pressure. 
We  include  the  spinal  ectopies  under  the  common  designation  of  "spina 


MALFORMATION'S,  ACQUIRED   DEFECTIVE   CONDITIONS     323 


bifida."  If  the  skin  over  the  hernia  of  the  spinal  cord  or  of  the  meninges  is 
present,  even  though  of  abnormal  appearance  (e.g.,  showing  hypertrichosis 
or  drawn  in  like  a  stellate  cicatrix),  we  speak  of  occult  spina  bifida  (see 
Fig.  91 )  ;  if  the  hernia  is  exposed,  however,  of  open  spina  bifida,  or  "Rachis- 
chisis"  (Fig.  92).  For  the  rest,  we  also  distinguish  here  the  simple  menin- 
goceles from  the  forms  which  contain  nervous  tissue.  The  spinal  analogue 
of  hydrencephalocele  is  called  myelocystocele.  The  most  pronounced  anom- 
aly, however,  is  presented  by  myelomeningocele,  in  which  the  spinal  cord, 
fissured  and  drawn  apart,  is  exposed  externally  and  closes  dorsally  the  summit 
of  the  meningocele  sac  as  the  so-called  "Zona  medullo-vasculosa."  Spinal 
ectopics  can  reach  the  size  of  a  child's  head.  They  are  usually  situated  in 
the  lumbo-sacral  region  which  corresponds  with  the  embryological  fact  that 
closure  of  the  spinal  canal  takes  place  from 
above  downward. 

Clinically,  in  so  far  as  there  is  not  occult 
spina  bifida,  the  tumor  naturally  first  strikes  the 
eye.  It  is  characterized  usually  by  definite 
fluctuation  and  partial  replaceability.  In  the 
last  test  great  caution  is  necessary  on  account 
of  the  danger  of  brain  pressure;  on  this  the 
fontanelles  are  often  noticed  to  protrude.  On 
the  other  hand,  when  the  child  cries  or  strains, 
the  tumor  sac  becomes  more  tightly  stretched. 

Myelomeningocele,  which  is  almost  always 
accompanied  by  serious  paralytic  symptoms, 
is  easily  to  be  recognized  by  the  deep  red  granu- 
lating "zona  medullo-vasculosa";  the  differen- 
tiation of  the  other  types,  even  with  the  as- 
sistance of  the  X-ray,  is  scarcely  possible 
before  the  operation.  Of  the  symptoms  of 
motor  involvement,  in  consequence  of  spina 
bifida,   symmetrica]   paralyses   in    the   legs   are 

next  to  he  mentioned.  Clubfeet  are  practically  always  present.  Many  times 
not  only  are  the  feel  and  legs  paralyzed,  but  there  is  complete  paraplegia — 
with  the  usually  deep  situation  of  the  tumor,  of  flaccid  character.  The  elec- 
trical reactions  may  he  lost,  the  patellar  reflexes  are  usually  markedly  reduced, 

the  Achilles  reflexes  are  generally  absent.  In  cervical  spina  bifida  I  have  also 
geen  spastic  paraplegia — a  very  ran'  occurrence.  Even  when  the  process  is 
located  high  up,  the  alius  usually  remain  free;  on  the  other  hand,  bladder  and 
rectal  paralyses  belong  to  the  typical  symptom-complex  of  spina  bifida.  In 
tin  ir  most  severe  form,  these  symptoms  are  found  in  the  spinal  hernias  situated 
low.  Disturbances  of  sensibility  air  usually  but  slight,  anil  only  exceptionally 
is  total  anesthesia  found  in  the  paralyzed  limbs.  Finally,  we  may  mention 
Certain  trophic  disturbances  (ulcers  on  tin-  genitals,  on  tin  heels,  over  the 
tumor  itself),  etc.,  here  and   there  also  perforating  ulcers  on  the   feet. 

Occult   spina  bifida   takes   a    special    position,   in    that    its   symptoms  develop 
only   in  late   childhood,  even    after   the    truth   year,     There   occurs,    then,    in 


Open  Spina  Bifida   (Cervical 
Meningocele). 


324  LECTURE    XXI 

previously  healthy  individuals,  little  by  little,  either  bilateral  pes  equinovarus, 
weakness  of  the  sphincters  appears,  which  can  increase  to  total  incontinence, 
or,  and  this  is  the  usual  thing,  both  these  morbid  conditions  develop  spon- 
taneously, accompanied  by  more  or  less  severe  pain  in  the  legs.  This  is  prob- 
ably a  result  of  the  traction  which  the  spinal  cord  fixed  at  the  point  of  the 
hernia  must  suffer  from  displacement  by  growth.  In  many  such  cases  lumbar 
hypertrichosis  (see  Fig.  91),  a  scarlike  depression  or  a  slight  bulging  of  the 
skin  in  the  lower  part  of  the  back  would  point  to  the  diagnosis  of  occult  spina 
bifida,  which  naturally  must  be  confirmed  by  X-ray  examination. 

The  "myelodysplasia"  of  Fuchs  can  be  considered  as  a  rudimentary  form 
of  occult  spina  bifida  under  which  is  understood  the  following  symptom- 
complex:  1.  Weakness  of  the  sphincters  which  leads  to  continued  nocturnal 
enuresis.  2.  Syndactylism,  or  membrane  formation  between  the  individual  toes. 
3.  Disturbances  of  pain  sense  in  the  toes.  4.  Anomalies  of  the  skin  and  ten- 
don reflexes  on  the  abdomen  and  in  the  legs.  5.  Occasional  deformities  of  the 
bones  of  the  foot,  eventually  combined  with  weakening  of  the  peroneal  mus- 
cles. 6.  Roentgenological  evidence  of  imperfect  closure  of  the  sacral  canal, 
defects  of  the  vertebral  arches,  etc.  7.  Exceptionally,  tropho-vasomotor  dis- 
turbances on  the  toes. 

Spina  bifida  is  often  complicated  by  other  malformations  and  defect  con- 
ditions, for  example,  by  hydrocephalus,  ectopy  of  the  bladder,  defects  of  the 
abdominal  walls,  etc.,  which  naturally  unfavorably  influence  its  prognosis. 
For  the  rest,  one  can  say,  the  greater  the  involvement  of  the  spinal  cord, 
the  worse  the  prognosis,  even  after  operative  procedures.  Without  operation 
the  outlook  is  very  bad ;  Wernitz,  in  90  patients  with  spina  bifida,  saw  only 
20  live  to  be  over  5  years  old;  the  majority  died  during  the  first  month  of 
life.  That  any  one  reaches  middle  age  is  excessively  rare.  Spontaneous  dis- 
appearance of  the  tumor  by  contraction  is  so  extraordinary  that  this  even- 
tuality need  not  be  reckoned  with ;  the  same  thing  applies  to  spontaneous  re- 
covery by  rupture  of  the  cyst.  The  last  accident  is  usually,  indeed,  fatal. 
If  the  cyst  ruptures  before  birth,  the  children  may  be  born  with  a  fistula 
which,  left  to  itself,  is  usually  sooner  or  later  the  port  of  entry  for  a  menin- 
geal infection.  Every  case  which  is  not  accompanied  by  other  serious  mal- 
formations  should  be  brought   under  surgical   treatment. 

C.      Congenital  Nuclear  and  Muscle  Defects 

In  the  cranial  nerves,  and  the  muscles  supplied  by  them,  congenital  ageneses 
are  not  so  very  rarely  observed.  Most  frequent  is  congenital  ptosis,  which 
may  be  uni-  or  bilateral.  Rarer  are  the  following  congenital  paralyses:  total 
external  ophthalmoplegia,  paralyses  of  looking  in  one  direction,  abducens 
paralysis,  paralysis  of  the  superior  rectus ;  facial  paralysis,  paralysis  of  the 
muscles  of  mastication  and  of  the  tongue ;  single  or  combined,  unilateral  or 
bilateral,  symmetrical  or  asymmetrical.  There  is  also  a  so-called  "congenital 
bulbar  paralysis."  For  these  absences  of  function,  as  anatomical  investigations 
have  shown,  there  may  be,  as  a  basis,  either  defects  of  the  muscles  concerned 
or  those  of  the  nuclei  of  the  cranial  nerves  supplying  them.      Now,  there  is 


MALFORMATIONS,  ACQUIRED  DEFECTIVE  CONDITIONS     325 

in  these  last,  however,  an  "infantile  nuclear  atrophy"  which  is  to  be  referred 
not  to  agenesis,  but  to  an  early  atrophy  of  these  structures;  these  two  forms, 
indeed,  are  in  principle  not  to  be  separated  from  one  another,  since  for  the 
early  infantile  atrophy  a  congenital  defect  should  be  a  prerequisite  also.  In 
the  paralyzed  muscles  the  electric  irritability  is  reduced  or  lost,  still  in  con- 
genital defects  reaction  of  degeneration  is  not  found. 

The  prognosis  of  all  these  conditions  is,  that  they  will  remain  stationary. 
The  relatively  slight  functional  disturbance  which  results  from  defects  of  the 
eye  muscles  is  noteworthy:  double  vision  scarcely  ever  occurs,  and  in  con- 
genital abducens  paralysis,  contrary  to  what  is  observed  in  the  acquired  form, 
there  is  usualty  no  contracture  of  the  antagonists  ;  the  eye,  on  account  of  this, 
remains  in  the  right  position  as  long  as  the  glance  is  not  turned  toward  the 
paralyzed  side.  Therapeutically,,  only  plastic  operations,  as,  for  example, 
transplantation  of  the  frontal  muscle  into  the  levator  palpebral  superioris, 
come  into  consideration. 

The  congenital  muscular  defects  on  the  trunk  and  extremities  are,  as  far 
as  anatomical  investigations  up  to  this  time  permit  a  decision,  always  of 
peripheral  nature,  that  is,  not  dependent  upon  ageneses  of  the  nuclei  in  the 
spinal  cord.  These  conditions,  which  Erb,  Damsch,  and  I  have  recognized, 
attract  clinical  interest  less  by  their  symptomatology  than  through  their  rela- 
tions to  progressive  muscular  dystrophy.  It  is  striking  that  the  muscle 
defects  are  observed  in  the  first  place  in  sucli  muscles  as  usually  atrophy 
frequently  and  early  in  progressive  muscular  dystrophy.  By  far  the  most 
frequent  is  defect  of  the  pectoralis,  which  Schlesmger,  among  54,000  patients 
of  one  Vienna  clinic,  found  5  times;  I  have  seen  6  cases  in  the  course  of  10 
years;  over  200  are  described  in  the  literature.  The  fact  that,  like  in  the 
Bcapulo-humeral  type  of  dystrophy,  the  clavicular  portion  of  the  pectoralis 
major  usually  is  preserved,  is  remarkable.  Although  rarer  than  isolated 
muscle  defects,  defects  of  whole  groups  of  muscles  also  occur;  also,  these 
affect  predominantly  such  muscle  complexes  as  arc  known  as  typical  localiza- 
tions of  the  dystrophies;  in  a  boy  observed  by  me,  for  example,  the  left  pec- 
toralis major  was  absent  (exceptionally,  in  toto),  also  the  triceps  brachii, 
a  pari  of  the  trapezius  and  the  rhomboids.  I  do  not  go  so  far  as  Erb,  and 
particularly  Damsch,  who  raise  the  question  as  to  whether  congeiiital  muscle 
defects  may  not  he  the  result  of  an  intra-uterine  variety  of  dystrophy.  It 
appears  to  me,  however,  that  between  the  total  inhibition  of  formation,  on 
the  one  hand,  and  the  congenital  predisposition  to  later  dystrophic  destruc- 
tion, there  is  only  a  difference  in  intensity.  Repeatedly  it  has  been  observed 
for  the  rest,  that  people  with  epngenital  muscle  defects  in  the  course  of  their 
later  lives  develop  progressive  muscular  atrophy.  Remarkable  is  the  excess- 
ively frequent  a cconipanimciit  of  congenital  muscle  defects  with  other  mal- 
formations of  all  sorts,  which  in  defects  of  the  pectoralis  are  usually  located 
about    the  shoulder  girdle,  thorax,  or  arm. 

It  is  also  striking  here  how  small  the  disturbances  of  function  are;  so,  one 
patient  with  left-sided  pectoralis  defect,  fenced  with  the  left  hand,  another 
shone  as  a  rider  and  swimmer.  These  are,  of  course,  examples  of  the  forma- 
tion of  vicarious  synergies  by  the   retained  muscles  or  portions  of  muscles. 


326  LECTURE    XXI 

Here,  also,  treatment  has  to  take  hold.  In  the  boy  above  mentioned,  by  fara- 
dization of  the  retained  portions  of  the  shoulder  girdle  and  by  suitable  exer- 
cises I  brought  him  to  the  point  of  being  able  to  execute  the  previously  im- 
possible, for  him,  "dip"  on  the  parallel  bars. 


LECTURE   XXII 

Infantile  Spastic  Hemiplegia  and  Diplegia;  Little's 
Disease ;  Idiocy 

Gentlemen:  We  will  to-day  review  a  number  of  disease  conditions  which 
etiologically  and  pathogenetically  quite  heterogeneous,  are  distinguished  by 
common  clinical  features.  These  are  always  the  result  of  injurious  factors 
which  have  affected  the  nerve  centers  either  prior  to  birth,  during  this,  or  in 
the  course  of  earliest  childhood,  and  have  led  to  spastic-paretic  symptoms  of 
hemi-  or  diplegic  type ;  the  syndromes  which  have  so  arisen  are  not  progressive, 
but  represent  either  a  stationary  residual  condition  or  even  manifest  tenden- 
cies to  spontaneous  improvement.  Within  these  rather  wide  nosological  limits 
we  include  as  "Little's"  disease  the  quite  frequent  cases  which  are  character- 
ized, 1,  by  diplegic  type,  and,  2,  by  the  so-called  Little's  etiology.  As  early 
as  1846  the  English  obstetrician  Little  had  pointed  out  the  predisposition 
which  premature  births,  multiple  births,  and  difficult  deliveries  seem  to  exert 
toward  the  development  in  the  children  in  question  of  bilateral  "spasticity  of 
the  limbs." 

In  order  to  introduce  some  system  into  this  rather  complicated  matter, 
I  must  first  make  you  acquainted  with  the  different  pathologico-anatomical 
bases  of  these  symptom-complexes.  I  will  divide  them  according  to  B.  Sachs, 
into  three  categories:  prenatal,  natal,  and  post-natal  lesions.  It  is  to  be 
remarked,  however,  that  in  individual  cases,  usually  not  the  autopsy,  but  a 
sufficiently  clear  and  extended  anamnesis  renders  possible  its  inclusion  in  one 
of    these    groups. 

1,     Prenatal  Lesions 

a.  Porencephaly. —  As  "true"  or  "primary  porencephaly"  we  denominate 
a  craterlike  depression  of  the  brain  surface  which  sinks  in  toward  the  ven- 
tricular cavity  to  communicate*  with  this  latter.  This  defect  arises  in  the 
intra-uterine  period  through  the  abnormal  depth  or  infolding  of  the  primary 
fissures  at  an  early  embryonic  period,  or  from  total  encephalitis  and  menin- 
gitis. These  last  sometimes  lead  also  to  "secondary"  or  "pseudo-porenceph- 
jtlies,"  cystlike  defects  of  substance,  in  consequence  of  the  shrinking  of  cica- 
trices on  the  convexity  of  the  brain.      Porencephalies  may  have  a  unilateral 

or    a    bilateral    symmetrical    location. 

b.  Lobar  Sclerosis. —  In  this  condition,  described  by  Virchow  as  "congen- 
ital encephalitis,"   there   is  a   gliotic   contraction  and   induration  of  the  totality 

827 


328  LECTURE    XXII 

or  of  the  greatest  part  of  one  or,  indeed,  of  both  hemispheres.  It  seems  to 
present  a  parallel  process  to  the  encephalomalacia  of  the  developed  brain. 
In  its  early  stage,  the  glia  tissue  remains  more  readily  preserved  after  processes 
leading  to  ischemia  and  hence,  after  destruction  of  the  nervous  parenchyma, 
can  proliferate  reactively,  and  after  that  contract.  The  diffuse  fetal  scleroses, 
hence  are  very  closely  related  to  the  prenatal  porencephalies,  but  owe  their 
origin  to  a  less  degree  of  intra-uterine  disturbance  of  the  blood  supply.  Lobar 
sclerosis  can  also  affect  only  one  or  both  halves  of  the  brain. 

c.  Tuberous  Sclerosis. — This  anomaly  was  formerly  considered  as  the  hy- 
pertrophic contrast  of  atrophic  lobar  sclerosis.  According  to  the  newest 
investigations  of  H.  Vogt,  however,  it  is  a  malformation  resembling  that  in 
tumors.  There  are  found  in  the  cortical  regions,  particularly  in  the  sensori- 
motor zone,  superficial  knotty  prominences  which  may  reach  the  size  of  a  nut 
and  consist  of  exceedingly  proliferated  glia.  Vogt  has  pointed  out  also  the  fre- 
quent finding  of  congenital  heart  and  kidney  tumors  (rhabdomyoma,  hyper- 
nephroma, liposarcoma),  as  well  as  congenital  adenoma  sebaceum  of  the  skin, 
in  patients  with  tuberous  sclerosis.  These  last  render  possible  the  diagnosis 
of  this  lesion  intra  vitam. 

(1.  Cysts  and  -foci  of  softening  in  the  brain  arise  from  the  causes  mentioned 
when  speaking  of  lobar  sclerosis,  rarely  before  birth;  where  this  is  the  case, 
obstruction  of  arteries,  usually  upon  a  syphilitic  basis,  are  to  be  held  respon- 
sible as  causes  for  the  circumscribed  destruction  of  brain  substance.  These 
are  usually  unilateral   lesions. 

e.  Spinal  foci  of  disease  w-hich  lead  to  descending  degeneration  of  the 
pyramidal  tracts ;  these  are  usually,  as  was  recognized  in  two  cases  anatom- 
ically examined  by  Dcjcrine,  also  of  syphilitic  origin. 

2.     Natal  Lesions 

These  furnish,  when  bilateral,  the  anatomical  substratum  of  Little's  disease. 

a.  Development  of  the  corticospinal  tracts  interrupted  by  premature  birth. 
This  defect  appears  in  many  cases  which  later  strikingly  improve  spontane- 
ously, capable  of  being  compensated  to  a  certain  degree  through  supplemen- 
tary development  of  these  tracts. 

b.  Hemorrhages  into  the  brain  substance,  or  upon  its  surface,  or  their 
residues  in  the  shape  of  cysts  and  areas  of  softening,  on  the  one  hand,  of 
adhesions,  with  the  meninges  on  the  other.  The  hemorrhages  occur  ( often 
bilaterally)  by  tearing  of  the  blood  vessels  in  a  difficult  labor  or  through  too 
sudden  passage  of  the  still  soft  skull  through  the  superior  strait  of  the  pelvis 
in  premature  delivery.  Factors  favoring  them  are  asphyxia,  twisting  of  the 
umbilical  cord,  brittleness  of  the  vessels  on  account  of  fetal  or  maternal 
diseases. 

c.  Cerebral  meningitis  and  encephalitis  post  partum,  which  may  occur  from, 
the  infection  of  hematomata,  in  case  the  abrasions,  bruises,  and  circulatory 
disturbances  which  the  skull  and  its  contents  suffer  in  difficult  labor,  do  not 
suffice  to  open  the  way  for  the  invasion  of  microorganisms.  The  results  of 
such  meningeal  infections  can  be,  among  other  things,  the  abnormal   folding 


INFANTILE    SPASTIC    HEMIPLEGIA    AND    DIPLEGIA       329 

of   the    cerebral   cortex   known    as   "microgyria,"   external,   or   even    internal, 
hydrocephalus,   as   well   as   pseudo-porencephalv. 

d.  Hemorrhages  into  the  spinal  cord  or  its  meningeal  envelopes.  These 
have  been  observed  after  breech  delivery  and  extraction,  after  turning,  etc. 

3.     Post  Natal  Lesions 

1.  Pseudo-porenccphalies  arising  from  embolic,  encephalitic,  and  meningo- 
encephalitic  processes,  also  through  trauma  (falls  on  the  head,  striking  it  on 
sharp  edges,  etc.). 

2.  Lobar  sclerosis;  3.  tuberous  sclerosis,  rarer  than  that  of  prenatal  ori- 
gin, but  still  agreeing  with  it  pathogeneticallv. 

■i.  Cysts  and  Foci  of  Softening. — These  changes  arc  relatively  frequentlv 
the  bases  of  spastic  diplegias  and  hemiplegias  arising  in  the  course  of  earliest 
childhood.  They  depend  upon  vascular  lesions  (rupture  or  thrombosis  of 
pathologically  altered  vessels,  embolism). 

5.  Acute  Polioencephalitis  of  Childhood  (Striimpcll). — The  relatively  rare 
localization  of  the  infectious  process  also  lying  at  the  base  of  acute  polio- 
myelitis, in  the  cerebral  cortex  already  considered  by  us  (see  Lecture  XVI, 
page  242),  occurs  unilaterally  and  leaves  behind  infantile  spastic  hemiplegia. 

Etiology 

Apart  from  the  last-mentioned  infectious  disease,  as  well  as  from  the 
already  mentioned  "Little's  etiology"  (premature  delivery,  multiple  births, 
pathological  delivery,  with  the  injuries  resulting  from  them)  specified  in  speak- 
ing of  the  natal  lesions,  congenital  syphilis  plays  the  chief  role*  among  the 
causes  of  infantile  spastic  hemi-  and  diplegia.  Most  of  the  just  sketched 
pathologico-anatomical  anomalies  can  even  at  the  autopsy  be  brought  info 
connection  with  existing  heredo-syphilis,  which,  on  account  of  the  well-known 
(i  ratogenic  action  of  the  luetic  virus,  on  the  one  hand,  and  its  injurious  effect 
upon  the  vessels  on  the  other  (endarteritis  syphilitica),  is  not  astonishing. 
That  luetic  patients  do  not  bring  into  the  world  hemiplegic  or  diplegic  children 
much  more  frequently  than  is  actually  the  case,  Sachs  explains  by  the  frequency 
of  abortion  in  such  families.  For  the  rest,  enough  cases  are  found  in  which 
there  have  been  a  number  of  abortions,  while  the  child  finally  carried  to  term 
presents  one  of  the  above-mentioned  prenatal  lesions.  In  the  prenatal,  as  in 
the  postnatal  forms,  Box  has  recognized  that  they  frequently  give  a  positive 
result  with  tin  "four  reactions"  (sec  above,  page  187).  Also  tuberculosis 
of  the  parents  is  not  rarely  found,  as  is  saturnism  and  alcoholism  of  the 
Ascendants.  In  congenital  cases  exhausting  diseases  of  the  mother  during 
pregnancy  can  often  lie  held  responsible.  There  is  often  a  history  of  physical 
oi-  psychical  trauma  which  has  been  suffered  by  the  pregnant  woman.  Value 
is  to  lie  placed  upon  these  anamnestic  statements  mainly  when  it  is  recognizable 
that    ;i    ^ro-s    trauma    has   affected    the   uterus.      Sachs   mentions   also    uremia   of 

•Indeed,  it  is  In  In-  held  responsible  for  many  cases  of  Little's  disease  (predisposition  to 
premature  delivery,  brittleness  of  tin'  vessels  in  luetic  foetuses). 


330  LECTURE    XXII 

the  mother.  First-born  children  are  predisposed  to  injuries  fit  birth  and  to 
Little's  disease.  On  the  other  hand,  as  Ganghofncr  and  Freund  have  shown, 
intra-uterine  injurious  factors  frequently  affect  the  last  children  in  a  large 
family  (exhaustion  of  the  maternal  organism  through  overexercise  of  the  gen- 
erative function). 

We  will  now  proceed  to  the  description  of  the  individual  forms  of  infantile 
hemi-  and  diplegia. 

A.    HEMIPLEGIA    SPASTICA    INFANTILIS 

(Unilateral  Form  of  Infantile  Cerebral  Palsy) 

The  great  majority  of  cases  arise  postnatally  during  the  first  year  of  life, 
and  usually  acutely  febrile  with  hebetude,  headache,  often  also  vomiting  and 
convulsions.  After  these  symptoms  pass  away  a  spastic  half-sided  paresis, 
usually  with  inclusion  of  the  face,  becomes  evident.  Generally  the  leg  is  less 
affected  than  the  arm;  the  patients  learn  to  walk  again  with  the  leg  slightly 
bent  at  the  knee,  adducted,  and  rotated  inward  with  the  foot  fixed  in  equino- 
varus  position;  the  latter  drags  and  is  "circumducted";  the  arm,  however,  and 
particularly  the  hand,  remains  incapacitated.  In  general,  the  same  tendency 
to  contracture  is  evident  in  it,  as  in  the  cerebral  hemiplegia  of  adults,  the 
arm  is  pressed  against  the  side,  flexed  at  the  elbow-joint,  and  there  is  pro- 
nation and  flexion  of  the  hand  and  fingers;  only  a  fixation  of  the  hand  in  a 
position  of  hypcrextension  is  sometimes  found.  Hypertonia,  exaggeration  of 
the  tendon  reflexes,  BabinskVs  phenomenon,  associated  movements,  etc.,  are 
naturally  present.  In  contradistinction  to  what  is  the  case  in  adults,  marked 
trophic  disturbances  usually  manifest  themselves  in  the  paralyzed  extremities: 
they  are  delayed  in  development,  the  X-ray  picture  shows  a  more  or  less 
marked  osteoporosis;  along  with  the  skeleton,  the  muscles  also  appear  atrophic, 
which  is  not  only  to  be  considered  as  a  result  of  inactivity,  but  as  a  "cerebral 
muscular  atrophy."  Reaction  of  degeneration  is  never  present.  Fingers  and 
toes  are  often  the  seat  of  athetoid,  more  rarely  of  choreic  movements  (see 
Lecture  V,  pages  80  and  85)  ;  unilateral  tremor  also  appears.  Sensibility  is, 
apart  from  atactic  disturbances  of  the  paralyzed  limbs,  usually  intact.  Apha- 
sia is,  even  with  paralysis  of  the  extremities  on  the  right  side,  very  rare,  which 
is  connected  with  the  early  age  of  the  patient.  The  patient,  early  deprived  of 
his  cerebral  cortex  on  the  left  side,  becomes  not  only  left-handed,  but  also 
learns  to  talk  with  his  right  hemisphere.  Accompaniment  of  hemiplegia  spas- 
tica infantilis  by  disturbances  of  intelligence  and  epilepsy  is  an  exceedingly 
common  occurrence. 

B.    DIPLEGIA    SPASTICA    INFANTILIS 

1.   Bilateral  Forms  of  Infantile  Cerebral   Palsy 

Among  these  forms,  according  to  Freud's  example,  3  types  may  be  dis- 
tinguished : 

a.  The  Bilateral  Hemiplegia  Type. — This  presents  a  doubling  of  the  in- 
fantile hemiplegic  symptom-complex  (in  which,  however,  differences  in  intensity 


INFANTILE    SPASTIC    HEMIPLEGIA    AND    DIPLEGIA       331 


between  right  and  left  usually  exist),  and  is  by  far  the  most  severe  variety 
of  infantile  cerebral  diplegia.  Also  bilaterally  innervated  cranial  nerves  can 
be  paralyzed,  on  which  account  pseudo-bulbar  paralytic  phenomena  (see  above, 
page  229)  occur.  The  arms  are  more  severely  affected  than  the  legs  and  the 
psyche  intensely  injured;  athetosis  is  frequent  (see  Fig.  93). 

b.   Paraplegic  Type. — The  majority  of  these  cases  are  congenital,  whether 
they  owe  their  origin  to  prenatal  lesions  or  are  to  be  referred  to  injuries  at 


Fio.  !>:!. 
Diplegia  Spastica    infantilis    ("Bilateral    Hemiplegic  Type";   Idiocy,   Athetosis). 

birth.      The   lasl    represenl    ;i    particularly   large   contingenl    of   the   cerebral 
form  of  Little's  disease. 

Many  cases  an'  noticed  even  in  nurslings,  since  when  dressing  them,  bathing 
them,  etc.,  the  legs  are  held  abnormally  --till'  and  immovable.  Usually,  how- 
ever, striking  disturbances  firs!  become  apparenl  when  the  child  should  begin 
to  take  his  firs!   steps.     It    is  then  noticed  that   the  thighs  are  held  in   forced 


332  LECTURE    XXII 

adduction  pressed  together,  or  indeed  crossed  like  a  pair  of  scissors,  the  knees 
are  moderately  flexed,  the  feet  in  equinovarus  position  with  the  toes  turned 
in ;  upon  attempts  at  walking,  the  knees  and  the  toes  rub  together.  The  legs 
are  decidedly  hypertonic,  and  oppose  great  resistance  to  passive  movements. 
If  the  child  is  placed  upon  a  chair  they  do  not  hang  down,  but  remain  more 
or  less  stretched  out  straight. 

As  the  child  grows  older,  these  disease  manifestations  not  rarely  improve, 
so  that,  indeed,  occasionally  in  the  6th  or  8th  year  of  life  locomotion  may 
be  nearly  normal.  Still  the  children  usually  long  retain  walking  on  the  toes 
and  a  slowed  and  laborious  rhythm  in  progression.  The  legs  are  delayed  in 
development  not  at  all,  or  only  inconsiderably,  in  contradistinction  to  the 
hemiplegic  form.  Indeed,  muscular  hypertrophy,  as  a  result  of  the  hyper- 
tonia, has  been  observed  (Ibrahim  and  others).  Exaggeration  of  reflexes, 
Babinski's,  often  also  Oppenheim's,  and  the  Mendel-Bechterew  symptoms,  are 
to  be  found  in  the  legs ;  on  the  other  hand,  the  arms  usually  remain  free  from 
every  anomaly,  or  they  are  only  slightly  affected  (somewhat  slow  movement, 
exaggeration  of  reflexes).  Sensibility  is,  as  a  rule,  intact;  on  the  other  hand, 
strabismus  and  weakmindedness   are  present. 

You  will  note  that  in  the  syndrome  just  sketched  the  paretic  symptoms 
are  much  less  manifest  than  the  hypertonic  ones.  Freud  sustains  with  good 
arguments  the  view  that  the  paralytic  symptoms  are  the  more  marked  the 
deeper  subcortical  the  hypertonic  phenomena,  the  more  decided  the  more 
superficially  cortical  the  lesion  lies.  No  wonder  that  the  "paraplegic  rigidity" 
is  so  preponderantly  frequent  in  Little's  birth-palsy,  which  usually  presents 
the  correlate  of  meningeal  hemorrhages.  Where,  however,  in  cerebral  spastic 
diplegia  in  childhood,  paresis  preponderates  over  hypertonia  (also  a  "para- 
plegic paralysis"  exists),  there  are  usually  deep-seated  prenatal  lesions. 

c.  "General  Rigidity." — This  also  usually  falls  under  the  "cerebral  cases 
of  Little's  disease."  All  four  extremities  are  affected;  the  arms,  however, 
much  less  than  the  legs ;  also  there  are  never  in  them  fixed  contractures,  as  in 
infantile  spastic  hemiplegias.  Hypertonia  considerably  preponderating  over 
the  paretic  phenomena  stands  in  the  foreground  of  the  clinical  picture.  Al- 
most always  disturbances  of  speech  and  intelligence  as  well  as  defect  symp- 
toms upon  the  part  of  different  cranial  nerves,  for  example,  strabismus,  are 
present. 

2.     Spastic  Spinal  Infantile  Paraplegia 

Here  belongs  chiefly  the  "spinal  type  of  Little's  disease,"  in  which  along 
with  the  characteristic  paraplegic  rigidity  of  the  legs,  no  other  anomaly  can  be 
found,  namely,  disturbances  of  intelligence,  strabismus,  speech  disturbances, 
choreic  or  athetoid  phenomena,  epilepsy,  etc.,  are  without  exception  absent. 
In  the  spastic  paraplegias  arising  prenatally  the  paresis  of  the  legs  stands  far 
in  the  foreground  as  compared  to  their  rigidity. 

Freud  assigns  to  infantile  palsy  also  cases  of  persistent  uni-  or  bilateral 
chorea  as  well  as  "double  athetosis"  in  early  infancy  (see  Lecture  V,  page 
86).      The  clinical  elements   of  hypertonia  and  paresis   are  here   replaced  by 


INFANTILE    SPASTIC    HEMIPLEGIA    AND    DIPLEGIA       333 

spontaneous    movements.      The    recommendation    to    record    these    forms    as 
'"infantile  cerebral  palsy  without  paralysis"  I  cannot  accept. 

Prognosis 

In  the  prognostic  estimation  of  the  spastic  paralyses  of  childhood,  it  is 
well  to  draw  a  distinction  between  the  motor  symptoms  and  their  eventually 
accompanying  symptoms  (among  which  the  psychical  anomalies  and  epilepsy 


Fig.  94. 
Diplegia  Spastica  Infantilis  ("Paraplegic  Kini<lit\,"  Feeble-Mindedness,  Speech  Disturbance). 

arc  full  of  significance).  These;  last  always  give  a  w\-y  poor  outlook  as  to 
Improvement  or  recovery,  epilepsy  having,  indeed,  a  tendency  to  grow  worse 
with  advancing  age,  and  its  appearance  being  threatened  up  to  adult  life, 
even  in  such  cases  which  at  first  do  not  present  this  complication.  The  hemi- 
plcgic  and  diplegic  symptoms^  <>n  the  contrary,  are  never  progressive,  fre- 
quently, indeed,  regressive.  Intact  intelligence  improves  the  prognosis  very 
Considerably,  since  treatment,  as  we  «ill  see,  cannot  dispense  with  the  coop- 
eration of  conscious  impulses  of  the  will  and  its  consequent  exercise  on  the 
part   of  the  patient.     Choreic  and  athetoid   phenomena   usually  persist,  but 


334  LECTURE    XXII 

the  patients  often  know  how  to  reckon  with  them  in  a  remarkable  manner, 
and,  in  spite  of  them,  use  their  limbs  for  all  sorts  of  acts.  Little's  and  the 
postnatal  forms,  particularly,  however,  "paraplegic  rigidity"  and  the  spinal 
form,  are  to  be  estimated  as  entirely  more  favorable  than  the  results  of  intra- 
uterine disease;  where  in  the  last  there  are  serious  defects,  death  usually 
occurs  during  the  first  months  of  life.  I  have  also  repeatedly  seen  children 
with  congenital  cerebral  palsy  develop  later  (at  the  time  of  puberty)  severe 
organic  nervous  diseases  or  die  (for  example,  with  fibro-sarcoma  of  the  lum- 
bar cord,  or  purulent  meningitis  of  the  convexity  of  obscure  pathogenesis). 
However,  the  prognosis  of  spastic  hemiplegia  and  diplegia  of  infancy,  as  to 
life,  is  favorable. 

Treatment 

A  causal  treatment  comes  under  consideration  in  a  large  number  of  cases 
of  uni-  and  bilateral  spastic  paralysis  in  children,  in  which  heredo-syphilis 
has  played  a  part.  Anti-syphilitic  treatment  should  be  begun  as  soon  as 
possible  and  should  not  be  carried  out,  as  is  so  often  the  case,  with  inade- 
quate methods.  The  sublimate  baths  (0.5  to  1.0  hydrarg.  bichloride  per 
bath)  preferred  by  many  pediatrists,  for  example,  are  at  most  of  use  as  an 
adjuvant.  Of  a  1  per  cent,  solution  of  iodide  of  mercury  (hydrarg.  biniodid, 
0.01;  sodii  iodid,  0.01;  sodii  chlorid,  0.08;  aq.  dest.,  10.00),0.2  to  0.5  cc,  is 
injected  every  second,  third  or  fourth  day  in  very  young  infants;  in  older  in- 
fants 0.5  to  0.1  cc  is  injected  every  second  day;  a  course  of  treatment  lasts 
6  weeks.  Internally,  hydrarg.  protiodid  or  calomel  can  be  given  in  milk;  the 
doses  are  for  the  first  three  months:  0.001  (gr.  %o)»  t.  i.  d. ;  for  the  rest  of 
the  first  year,  0.003  to  0.006  t.  i.  d.  (gr.  %o  to  gr.  Vw).  For  the  second 
year  0.0075,  for  the  third  0.01,  t.  i.  d.  As  a  substitute  for  the  inunction  treat- 
ment Bruno  Block  recommends  the  "plaster  treatment"  as  very  simple:  one 
extremity  or  a  correspondingly  large  portion  of  the  trunk  is  covered  with 
mercurial  plaster.     The  plaster  is  left  on  for  a  week. 

In  cases  beginning  with  fever  during  the  early  years  of  life  antiphlogistic 
treatment  is  in  place  as  long  as  the  acute  stage  lasts  ;  ice  bag  to  the  head,  free 
purgation  by  several  doses  of  calomel,  eventually  the  application  of  leeches 
behind  the  ears.  For  the  convulsions,  enemata  of  the  following  composition 
may  be  given  with  advantage:  Chloral,  hydrat.,  0.4  (gr.  vii)  ;  pulv.  camphorae, 
0.1  (gr.  ll/2);  vitell.  ovi   I;  aq.  dest.,  q.  s.  ad  200.0   (5  vi). 

As  to  the  causal  therapy  of  the  initial  stage  of  Little's  disease,  the  operative 
removal  of  meningeal  hematomata  after  protracted  labors  has  been  undertaken 
by  Cushing  and  other  surgeons:  the  children  did  not  develop  Little's  disease 
(perhaps  they  would  not  have  done  so  anyhow). 

The  treatment  of  the  later  stages  in  all  spastic  hemiplegias  and  diplegias 
of  childhood  is  above  everything  else  by  curative  gymnastics  and  exercise 
therapy.  All  the  measures  to  be  applied  by  neurologists,  orthopedists  and  sur- 
geons have  the  one  end  of  shaping  the  conditions  for  the  regression  of  the 
spastic  resistance  and  the  compensation  of  the  paresis  as  favorably  as  possible. 
As  already  said,  what  may  be  expected  from  our  therapeutic  endeavors,  apart 
from  the   seriousness   of  the  lesion   depends,  above  everything  else,  upon  the 


INFANTILE    SPASTIC    HEMIPLEGIA    AND    DIPLEGIA       335 

mental  level  of  the  patient.  At  the  start  we  have  to  place  our  chief  emphasis 
upon  the  application  of  physical  agents  (electricity,  massage,  hot  baths)  ;  as 
far  as  electricity  is  concerned,  the  rubbing  the  hypertonic  muscles  with  the 
anode  of  the  galvanic  current  often  acts  quite  favorably.  For  this  application 
a  rather  large,  flat  electrode  is  selected  and  all  sudden  variations  of  current 
strength  which  may  act  as  irritants  are  to  be  avoided  by  careful  use  of  the 
rheostat;  intensity  3  to  5  milleamperes.  Those  muscles,  on  the  other  hand, 
which  are  not  greatly  hypertonic  but  paretic,  can  be  treated  by  the  labile  ap- 
plication of  the  cathode;  only  one  should  limit  himself  to  such  strength  of 
current  as  suffices  to  produce  a  definite  contraction  on  cathodal  closing.  On 
the  other  hand,  I  avoid  in  general  the  faradic  current,  since  with  it  not  only 
the  muscles  which  it  is  desired  to  influence,  but  also  those  tending  to  con- 
tracture are  affected  by  diffusion  of  the  current.  As  to  bathing,  according  to 
Heubncr,  hot  baths  are  much  to  be  recommended:  3  to  4  times  a  year  during 
a  period  of  4  to  6  weeks,  a  bath  lasting  for  10  to  15  minutes  is  given  every 
dav;  beginning  with  a  temperature  of  37  C.  (98.6°  F.)  and  gradually  raising 
it  to  40  C.  (  104"  F.).  Also  the  use  of  natural  hot  baths,  especially  those  of 
higher  temperature,  for  example,  Baden-Baden,  Aix-les-Bains,  Teplitz,  Hot 
Springs  of  Virginia,  Arkansas  and  California  are  indicated.  Massage  can  be 
carried  on  in  the  bath;  it  consists  in  careful  (not  jerky)  stretching  of  the 
spastically  shortened  muscles  whose  hypertonia,  as  Hoffti  has  shown,  can  be 
diminished  by  tapotement  of  the  ends  of  the  tendons;  their  antagonists,  on  the 
other  hand,  art'  stroked  and  kneaded. 

It  quite  frequently  occurs  that  the  systematic  exercise  of  active  movements, 
which  must  be  begun  as  soon  as  the  intelligence  of  the  child  permits  it,  must 
be  preceded  by  orthopedic  measures;  above  everything  tenotomy,  in  its  modern 
modifications  (oblique,  stair-like  incisions),  with  application  afterward  of  suit- 
able bandages,  splints,  etc.,  also  shortening  of  tendons,  tendon  transplanta- 
tions, etc. 

On  the  other  hand  osteotomy  of  the  thigh  below  the  trochanter,  which  has 
been  recommended  in  Little's  disease  with  the  view  of  moving  the  center  of 
gravity  of  tin  body  farther  backward,  appears  to  have  little  justification. 
Fiirxtcr'x  operation  ( rhizotomia  posterior)  has  met  with  considerably  more 
encouragement.  In  it,  a  number  of  posterior  spinal  roots  corresponding  to 
tin  spastic  muscles  are  cut  in  order  (according  to  the  mechanism  explained  in 
Lecture  I,  page  7)  to  obtain  relaxation  of  the  hypertonia.  Like  all  other 
operations,  however,  this  last  also  promises  success  only  with  exceedingly  care- 
ful after-treatment,  long  and  continuous  gymnastics  and  exercise  therapy,  in 
Connection  with  which  appliances  like  the  resistence  apparatus  of  Zander  & 
Herz,  etc.,  can  find  frequent  application. 

(Supplementary.)  The  Idiocies. 

We  have  seen  how  often  infantile  cerebral  paralyses  are  accompanied  by 

idiocy.  This  last  can  also  occur  without  any,  or  witli  such  slight  paralytic 
manifestations  that  it  entirely  dominates  the  picture.  The  accurate  presen- 
tation of  its  semiological  peculiarities  belongs  to  the  domain  of  psychiatry; 


;33G 


LECTURE    XXII 


I  would,  however,  sketch  broadly  the  psychic  relations  common  to  all  forms 
of  idiocy  and  give  you  also  some  insight  into  their  most  important  clinical 
varieties. 

Under  idiocy  in  the  broad  sense  are  included  the  conditions  of  psychical 
arrest  of  development  characterized  by  want  or  defectiveness  of  the  intellectual 
functions ;  the  term  "idiocy"  in  the  narrower  sense  is  reserved  for  those  severe 
forms  in  which  the  individual  is  unable  to  direct  his  own  life  within  the  bounds 
of  society.  In  the  most  extreme  cases  of  this  sort  all  ability  to  receive  im- 
pressions and  to  form  conceptions  is  absent ;  there  is  "mind  blindness"  and 
"mind  deafness."      The  speech  does  not  even  reach  rudimentary  development, 

in  short,  the  mental  level  is  con- 
siderably below  that  of  the  higher 
mammals.  On  the  other  hand,  how- 
ever, the  defective  condition  may  be 
slight,  so  that  a  smaller  or  larger 
number  of  concepts  develop,  a  cer- 
tain amount  of  education  is  possible 
by  a  rational  way  of  bringing  up, 
ability  to  speak  is  developed  to  a 
greater  or  less  extent,  etc.  There 
are  the  most  manifold  gradations 
until  the  slighter  grades  which  are 
denominated  "imbecility"  are  reached 
(in  this  latter  the  mental  condition, 
in  contradistinction  to  idiocy,  per- 
mits the  exercise  of  some  calling,  the 
individual  can  still  be  designated  as 
"intra-social"),  and  to  the  very 
slightest  "debility"  or  feeble-minded- 
ness,  whose  separation  from  physio- 
logical stupidity  is  quite  indefinite. 
Frequent  accompaniments  of 
idiocy  are:  Epilepsy  (in  about  one- 
third  of  the  cases)  ;  genital  infantil- 
ism ;  physical  stigmata  of  degenera- 
tion (Gothic  palate,  asymmetry  of 
the  skull  =  oblique  skill,  plagiocephaly,  "pithccoid"=ape-like  formation  of  the 
face  (see  Fig.  95),  anomalies  of  the  teeth,  hare-lip,  cleft  palate,  prognathism, 
hyperdactylism,  syndactylism,  malformations  of  the  ears,  etc.)  ;  ambidextrism 
(in  about  one-sixth  of  the  cases);  backwardness  in  development  of  the  body, 
automatisms  of  movement  (showing  the  teeth,  boring  the  fist  into  the  mouth), 
grimacing,  rocking  the  body,  etc.)  ;  reduction  of  pain,  temperature  and  muscle 
sense.  According  to  their  conduct  with  regard  to  the  external  world  idiots  are 
divided  into  apathetic  (anergetic  or  torpid)  and  erethristic  (energetic,  agile 
or  versatile). 

All  the  pathologico-anatomical  lesions  mentioned  on  speaking  of  infantile 
cerebral  palsy  when  they  affect  the  mechanisms  of  the  mental  functions,  furnish 


Pithecoid  Idiot. 


IDIOCY  337 

the  substratum  for  more  or  less  profound  idiocies  as  do  also  malformations  of 
the  brain,  like  true  poreneephali,  or  focal  diseases  in  their  earlier  period  of 
development,  cysts,  foci  of  softening,  pseudo-porencephaly,  further,  micro- 
gyria depending  upon  meningitis,  hypertrophic  tuberosis  and  atropine  lobar 
sclerosis.  Other  malformations  and  arrests  of  development  of  the  brain  found 
in  idiots  are,  among  other  things,  defect  of  the  corpus  callosum  and  Sachs' 
"Agenesis  corticalis,"  in  which  the  cortex  may  appear  macroscopically  normal, 
the  cortical  ganglion  cells,  however,  prove  to  be  rudimentary.  "Hydrocephalic 
idiocy"  we  have  already  considered  in  the  preceding  lecture.  There  remains 
to  us  now  the  task  of  going  over  a  few  forms  of  idiocy  particularly  marked 
by  their  physical  accompanying  symptoms. 

Amaurotic  Idiocy 

This  is  a  marked  family  disease  which  was  recognized  in  its  clinical  peculi- 
arities in  188T  by  the  New  York  neurologist,  B.  Sachs,  although  six  years 
earlier  the  English  ophthalmologist,  Warren  Tay,  first  saw  and  described  the 
alteration  of  the  eye  grounds  pathognomonic  for  this  affection.  On  this  ac- 
count it   is  usually  spoken  of  as  the  "Tay-Sach's"  disease. 

In  typical  cases  at  an  age  of  from  3  to  6  months  an  infant  up  to  this 
time  normal,  is  affected  by  a  torpor  increasing  until  at  length  he  lies  almost 
continuous] v  in  a  condition  of  complete  apathy,  relaxation  and  immobility,  in 
which  however,  breathing,  the  heart  beat,  and  the  taking  of  nourishment  is 
.still  undisturbed.  All  the  muscles  are  hypotonic,  if  the  child  is  set  upright  the 
head  rolls,  without  support,  in  all  directions.  Now,  however,  spastic  phenomena 
become  more  and  more  mixed  with  this  picture  of  hypotonic  akinesia  and  fi- 
nally occupy  the  foreground.  At  first  they  are  intermittent,  tonic  extension 
Spasms,  finally  a  continual  spastic  condition  in  the  place  of  the  former  relaxa- 
tion. Now  the  nutrition  suffers  through  involvement  of  the  swallowing  and 
sucking  mechanisms  and  death  occurs  after  skeleton-like  emaciation.  It  can 
be  stated,  as  a  rule,  that  this  occurs  before  the  completion  of  the  second  year. 
Only  once  did  such  a  child  live  to  be  8  years  "Id. 

Parallel  with  the  progressive  psychic  and  motor  disturbances  proceeds  the 
lo^s  of  vision  with  a  peculiar  alteration  of  the  eye  grounds;  this  is  a  clouding 
of  the  retina  distributed  symmetrically  in  both  fundi  surrounding  the  yellow 
spot,  of  whitish  color  hut  with  a  cherry-red  point  in  the  middle  (sec  Fig.  96). 
Finally,  optic  atrophy  is  added  to  it. 

It  is  noteworthy  that  the  typical  cases  practically  always  occurred  in 
Jewish  families  originating  in  Poland,  which  raises  the  suspicion  of  descent 
from  a  far-removed  common  ancestor.  According  to  Apert's  collection,  only 
two  of  the  166  cases  which  he  recognized  as  undoubted  instances  of  the  disease 
formed  an  exception  to  this  rule.  It  is  paradoxical,  however,  that  the  over- 
whelming majority  of  cases  of  amaurotic  family  idiocy  have  not  come  under 
observation  in  Poland,  bul  among  Polish  dew  emigrants,  and  for  the  most  part 
in  America,  though  also  in  England,  Germany,  Austria.  France,  and  even  in 
Australia.       Perhaps    the   "transplantation"   into   other  conditions   of  lit','    is   an 

■zciting  cause.     The  atypical  cases  In  which  the  characteristic  macular  altera- 


338 


LECTURE    XXII 


tions  are  absent  (here  belong  also  the  so-called  "late  form"  described  by  Spiel- 
meyer  and  Vogt),  do  not  manifest  this  ethnological  predilection,  or  only  to  a 
very  small  degree. 

.  The  pathological  anatomy  of  the  Tay-Sach's  disease  is  very  accurately 
known.  All  the  gray  matter  of  brain  and  spinal  cord  shows  marked  cytological 
alterations  (swelling  of  the  ganglion  cells,  disappearance  of  the  Nissl  granules 
and  of  the  fibers  passing  through  the  cell  body,  vacuolation,  etc.).  Also  the 
retinal  cells  of  the  macula  and  its  neighborhood  are  affected  by  the  degenerative 


Eye-ground  in 


Fig.  96. 
Amaurotic   Idiocy. 


(After  Sachs.) 


process,  through  which  they  become  opaque.  Hence  the  white  circle  about  the 
fovea  centralis.  Only  in  the  last  (which  has  no  ganglion  cells)  the  choroid  is 
visible  afterward  as  before,  and  by  contrast  imposes  itself  as  Tay's  "cherry- 
red  spot."  As  chemical  correlative  of  the  disappearance  of  the  tigroid  sub- 
stance, that  of  nucleo-proteid  in  the  whole  nervous  system  has  been  shown,  so 
it  is  probably  a  constitutional  disease  of  metabolism  of  the  ganglion  cells. 


Microcephalous  Idiocy 

This  form,  on  account  of  the  abnormal  smallness  of  the  skull,  takes  on  a 
particularly  characteristic  picture.  We  must  distinguish  true  microcephaly 
(simple  pure  microcephaly,  micro-encephaly)  from  pseudo-microcephaly.  At 
the  base  of  the  former  there  lies  a  genuine  hypoplasia  of  the  brain ;  in  the 
second,  the  inhibition  of  growth  and  development  of  the  brain  is  caused  by 
gross  intra-uterinc  brain  diseases.     In  any  case,  the  anomaly  of  the  skull  forms 


IDIOCY 


339 


the  secondary  correlative  of  an  abnormal  condition  of  the  brain.  Virchow's 
hypothesis,  according  to  which  premature  synostosis  of  the  sutures  of  the 
skull  is  responsible  for  the  arrested  development  of  the  brain,  has  long  been  aban- 
doned. With  it  also,  fortunately,  the  "Lane-Lannelongue"  operation  in  which 
by  removal  of  segments  of  bone  from  the  roof  of  the  skull  or  even  the  separa- 
tion of  the  calvarium  (craniamphitomie)  the  compressed  brain  could  be  fur- 
nished relief.     Fig.  97  represents  a  plaster  model  of  one  of  the  most  celebrated 


Fig.  97. 
Microcephalous  Idiot.     (Modeled  after  Life.) 

cases  of  family  true  microcephaly,  which  is  preserved  in  the  Pathological  In- 
stitute at  Basle.  The  peculiar  form  of  lace  which  is  shown  in  our  picture  has 
led  to  the  designation  "Bird  head"  or  "A/tec  Type."  The  lowest  brain  weights 
observed  were  15.9  grin,  in  a  7  wicks'  old  boy;  288  gin.  in  a  -Hi  years'  old 
woman.  Microcephaly  must  not  be  confused  with  the  "nanocephaly"  which 
Occurs  in  small  individuals.         ' 


Mongoloid  Idiocy 

As   the  most   important   clinical   peculiarities  <>f  this   form   of  Idiocy   dis 
covered  by  Down  in   lH(>f>,   the  following  group  of  symptoms  may   be  men- 
tioned.    First,  the  peculiar  physiognomy  which  lias  given  the  disease  its  name, 
tin'  "Tartar"  or  "Kalmuck"  type  (see  Fig.  98);  flal   face,  wide-bridged  nose. 
prominent    cheek  bones,  almond   eyes   (often   with  epicanthus),   with    reddened 


340 


LECTURE    XXII 


lids  and  margins,  and  without  lashes,  a  grayish-brown  complexion,  with  red 
cheeks  which  produce  the  impression  of  being  painted  like  a  clown,  round  skull, 
flattened  at  the  back.  Then  the  hypertrophy  of  the  tongue  with  enlargement 
of  the  circumvallate  papilla*  and  remarkable  wrinkling  of  the  surface  ("scrotal 
tongue").  Abnormal  flaccidity  and  softness  of  the  muscles,  ability  to  bring 
the  joints  into  abnormal  positions,  like  a  "snake  man."  Often  there  are  con- 
genital anomalies  of  the  internal  organs  (atresias,  umbilical  hernia,  congenital 
defects  of  the  heart,  arrests  of  development  of  the  teeth,  indications  of  dwarf- 
ism with  normal  ossification  shown 
by  the  X-ray,  but  with  atrophy  of 
the  end  and  shortening  of  the  mid- 
dle phalanx  of  the  little  finger, 
more  rarely,  a  tendency  to  partial 
giantism). 

The  growth  of  hair  is  not 
affected,  the  eyebrows,  indeed,  are 
abnormally  heavy.  The  defect  in 
intelligence  in  "Mongoloids"  is, 
as  a  rule,  combined  with  quite 
considerable  ability  to  fix  the  at- 
tention and  reactibility,  with 
happy  mood  and  a  great  ten- 
dency to  imitation.  This  exceed- 
ingly characteristic  form  of  idiocy 
is  not  very  rare  in  our  neighbor- 
hood; Konrad  Fret/  found  in  the 
Aargau  Idiot  Asylum  at  Biber- 
stein,  among  60  inmates,  3  Mon- 
goloids ;  in  England  and  Scan- 
dinavia also  5  per  cent,  are  found, 
in  Germany,  only  2  per  cent.,  on 
the  other  hand,  according  to 
Kowalewsky,  in  the  Government 
of  Petersburg,  the  percentage 
among  the  inmates  of  asylums  is 
10,  in  the  Government  of  Kazan, 
indeed,  25.  Whether  this  last  is  connected  with  the  decided  Mongol  admixture 
in  the  population  cannot  be  certainly  stated. 

According  to  H.  Yogt,  Mongolism  is  connected  with  retardation  in  the 
later  stages  of  development  of  the  brain ;  according  to  Buschan,  Weygandt, 
Fret)  and  others,  it  could  be  attributed  primarily  to  disturbances  of  internal 
secretion  (thymus?).  The  brain  is  often  small,  sometimes  it  presents  defects  in 
development  (for  example,  partial  defect  of  the  corpus  callosum).  Often  the 
convolutional  type  renders  recognizable  an  abnormally  simple  development  with 
very  coarse  and  wide  convolutions ;  the  cortical  cells  are  imperfectly  differen- 
tiated, the  cortex  abnormally  rich  in  vessels. 


Fig.  98. 
Mongoloid  Idiot. 


CRETINISM  341 

Cretinistic  Idiocy 

Those  forms  of  idiocy  which  occur  in  connection  with  alteration  of  the 
thyroid  gland  or  of  its  "internal  secretion"  and  are  accompanied  by  peculiar 
changes  in  the  skeleton,  demand  particular  attention.  We  include  them  under 
the  common  designation  of  "Cretinism."  It  may  be  remarked  here  that  in  the 
earlier,  and  unfortunately  also  in  the  newer  literature,  much  confusion  has 
been  produced  by  bringing  together  under  this  designation  different  diseases 
of  other  sorts  (running  their  course  partly  with  and  partly  without  idiocy) 
on  account  of  superficial  resemblances  in  their  external  appearance.  Above 
everything  the  just  described  "Mongolism,''  further,  fatal  chondrodystrophy 
or  achondroplasia,  a  form  of  disproportioned  dwarfism  (micromelia)  depend- 
ent upon  a  congenital  defect  of  the  zones  of  direction  of  the  cartilages  of  the 
skeleton,  which  does  not  imply  any  disturbance  of  the  intelligence  or  any  al- 
teration of  the   thyroid. 

We  distinguish  1,  sporadic,  and  2,  endemic  cretinism.  The  first,  again, 
occurs  in  two  forms:   a,  infantile  myxcedema,  and  b,  thyreoaplasia  congenita. 

We  will  become  acquainted  in  the  lecture  after  the  next  with  the  disease 
picture  of  myxoedema  which  occurs  from  the  destruction  or  the  serious  inter- 
ference with  the  function  of  the  thyroid  gland  and  in  which  the  mental  functions 
are  more  or  less  seriously  affected.  If  this  myxoedema  occurs  in  childhood 
there  results  besides  this,  a  retardation  in  the  growth  of  the  skeleton,  a  pro- 
portioned dwarfism  on  account  of  injury  of  the  cartilage,  the  bone  marrow 
and  the  periosteum,  through  which  infantile  myxoedema  takes  on  the  picture  of 
a  sporadic  cretinism. 

Similar,  only  much  more  marked,  are  the  anomalies  in  congenital  defect  of 
the  thyroid  gland  (thyreoaplasia).  Here  also  the  hypothyreotic  pathogenesis 
of  idiocy  is  very  plain. 

More  complicated  relations  exist  in  endemic  cretinism  which  is  at  home 
in  such  regions  in  which  goiter  and  deaf-mutism  occur  in  great  frequency. 
The  Alpine  and  sub-Alpine  valleys  are  afflicted  with  this  severe  scourge.  In 
Switzerland,  in  Tyrol,  Styria,  Savoy,  Piedmont,  in  the  Veltlin.  For  Swit- 
zerland II chinch  and  Kikjcii  liirchcr  have  established  the  striking  fact  that 
there  is  a  connection  between  cretinism  and  the  geological  formation  of  the 
soil,  that  above  everything  the  marine  formations  of  the  1'aleozoic,  the  Triassic 
and  tin1  Tertiary,  are  affected,  while  all  the  fresh-water  formations,  also  the 
eruptive  and  the  crystalline  rucks  and  the  sediments  of  the  Jura  and  the  lower 
chalk  remain  i'rvv  from  endemic  goiter,  cretinism  and  deaf  mutism  (  /■;.  liirchcr 
ela-ses  these  .'}  conditions  together  as  cretinistic  degeneration).  That  the 
cause  is  to  lie  sought  iii  the  drinking-water  is  certain:  for  instance,  it  has  been 
possible  to  check  the  endemic  cretinistic  degeneration  in  the  Canton  Aargau 
by  supplying  the  villages  of  the  endemic  district  with  water  from  healthy 
localities.  Further,  in  the  rare  endemics  in  lowlands  (for  example,  on  the 
island    of   Seliutt,    or   along   the   course   of   the    Mur    in    Hungary)    it    has    been 

found  that  the  affected  regions  get  their  water  from  rivers  which  rise  iii  cretin 
regions.     The  nature  of  the  disease  agenf  and  its  method  of  action,  which  for 

the   rest    presupposes   a    pergonal    predisposition,   on    the   other   hand   are   nol    \el 


342 


LECTURE    XXII 


explained,  in  spite  of  recent  hypotheses.  In  no  case  can  endemic  cretinism  in 
its  whole  clinical  picture  be  referred  to  a  pure  thyreoaplasia  or  hypothyreosis. 
The  injurious  agent  must  primarily  have  other  points  of  attack  than  the  thy- 
roid gland,  since  the  symptom-complex  is  a  much  more  complex  one  than  in 
both  the  varieties  of  sporadic  cretinism,  and  the  reaction  to  thyroid  adminis- 
tration, as  we  will  soon  see,  is  scarcely  ever  appreciable. 

The  weakmindedness  can  reach  an  .excessive  degree:  there  are  cretins  who 
can  never  be  taught  to  take  nourishment  for  themselves,  but  must  even  be  fed 
with  the  tube.  Along  with  these,  however,  there  are  often  slighter  forms  of 
idiocy,  imbecility,  and  debility  (feeblemindedness).     The  apathetic  torpid  form 


of  idiocy  predominates.  There  are,  however,  very  disturbed,  obstinate  and 
troublesome  cretins  who,  as  the  accomplished  describer  of  Alpine  cretinism, 
Peter  Rosegger,  expresses  it,  "are  capable  of  the  7  deadly  sins."  Where  they 
can  learn  to  talk  it  is  imperfect,  stammering.  Smell,  taste,  sensibility,  and 
especially  hearing,  are  more  or  less  seriously  affected;  vision,  on  the  contrary, 
is  usually  good.  The  head  is  usually  abnormally  large,  more  rarely,  micro- 
cephalous, frequently  asymmetrical.  The  nose  is  wide,  deeply  drawn  in  at  the 
loot,  the  eyes  are  widely  separated  from  one  another,  small,  and  slit-like; 
especially  is  the  whole  face  widened.  A  low  forehead,  a  dry,  wrinkled  skin  of 
an  unclean  color,  sparse  or  absent  growth  of  beard,  a  wide  mouth  with  irreg- 
ular teeth,  a  short  neck  on  which  a  goiter  is  often   prominent,  complete  the 


CRETINISM  343 

grotesque  physiognomy  (see  Fig.  99).  The  body  structure  is  characterized 
in  general  by  disproportioned  dwarfism,  the  lack  of  development  in  length 
affects  chiefly  the  lower  extremities,  but,  however,  never  reaches  the  excessive 
degree  observed  in  achondroplasia;  the  trunk  is  not  only  too  long  in  propor- 
tion to  the  limbs,  but  it  is  usually  very  massive  and  plump,  with  the  exception  of 
the  sometimes  deformed  pelvis.  Very  many  cretins  are  the  bearers  of  great 
inguinal  hernias.  In  the  X-ray  picture  an  irregular  delay  in  the  formation 
of  bone  nuclei  and  persistence  for  a  long  time  of  the  epiphyseal  cartilages 
(until  the  middle  of  the  third  decade)  is  noted. 

Along  with  these  typical  forms  there  are  in  cretin  countries  a  great  many 
cases  in  whom  there  are  only  a  small  number  of  the  anomalies  mentioned:  they 
-are  called  "cretinoids,"  or  "half  cretins.'' 

The  Treatment  of  Idiocy 

A  causal  treatment  is  only  possible  in  idiots  where  there  is  either  congenital 
svphilis  or  where  thyreogenic  causes  prevail.  In  the  last  there  is  a  great 
difference  between  endemic  cretinism  anil  the  sporadic  forms,  however.  In- 
fantile myxcedema  reacts  even  in  the  psychical  condition,  often  with  surpris- 
ing improvement,  even  recovery,  to  the  administration  of  thyroid  gland  prepa- 
rations, for  example,  the  thyroid  gland  tabloids  of  Burroughs,  Wellcome  & 
Co.,  or  the  Thyraden  tablets  of  Knoll  (each  containing  0.3  grm  of  the  thyroid 
gland  substance  of  the  sheep  or  of  the  hog,  per  tablet).  The  children  are  given 
one-half  to  one  tablet  (that  is,  0.15  to  0.3  gland  substance)  per  day.  In 
thvreoaplasia  improvement  is  the  rule  under  organo-therapeutic  treatment, 
but  it  is  usually  less  marked  than  in  infantile  myxcedema  and  there  is  practi- 
cally never  recovery.  Endemic  cretinism  is  still  less  suitable  for  thyroid  treat- 
ment. In  slight  cases,  indeed,  Magnus-Levy  and  Wagner  v.  Jauregg  have 
Obtained  improvement,  but  this  almost  always  fails  to  appear.  The  transplan- 
tation of  viable  thyroid  into  the  spleen  of  a  cretin  as  I'nj/r  has  undertaken,  has 
only  a  certain  influence  in  encouraging  growth,  none  upon  the  psyche.  The 
pedagogic  treatment  of  psychical  defect  conditions  of  every  pathogenesis  is 
only  hopeless  in  those  of  the  highest  degree  and  accomplishes  indeed,  in  the 
majority  of  cases,  as  among  others,  Boumeville,  has  shown  upon  the  enormous 
NKiti  rial  of  the  Bicetre  Asylum  at  Paris,  satisfactory  and  encouraging  results. 
The  instruction  of  idiots  has  become  a  scientific-ally  well  founded  specialty. 
In  order  to  develop  the  muscular  sense  in  these  children  we  usually  begin  by 
pstructing  them  In  the  use  of  their  extremities  (by  the  aid  of  their  faculty  of 
imitation)  for  all  actions  of  daily  life  (also  eating,  etc.).  At  4  or  5  years  the 
education  proper  begins,  which  firs!  has  to  render  of  use  those  capabilities 
Much  have  remained  least  rudimentary,  also  the  instincts  present  (lor  e\ 
ample,   the  desire    for   lid  hits),   are  utilized   for   the   accomplishment   of  results. 

for  fixing  the  attention,  etc.      All    this   can   only   be   ace plished   iii   a    way 

promising  results   In   an   institution.      On   this  account    idiots  should   in  all   cases 

Ee  removed  from  home  in  the  5th  year.  With  tireless  and  intelligent  manage- 
ment a  large  part  of  them  learn  in  time  to  read  and  write  and  many  of  them 
can   in  any  case  learn   to  employ  themselve8  as   helpers   in   healthy   manual  occu- 


344  LECTURE    XXII 

nations  (farming,  gardening,  etc.).  In  Basle  the  endemic  cretins  have  to  such 
an  extent  a  monopoly  of  the  peddling  of  sand  that  the  terms  "Sand-mannli" 
and  "Sandwybli"  (little  sand  men,  little  sand  women)  in  the  popular  speech 
are  equivalent  to  "cretin,"  and  they  are  able  to  carry  out  this  modest  but 
useful  occupation  with  entire  satisfaction.  Further  mental  development  of 
idiots  is  terminated  only  at  about  20  years. 


LECTURE  XXIII 

Dysglandular  Symptom-Complexes 

Gentlemen  :  We  will  occupy  ourselves  in  this  lecture  with  several  disease 
pictures  for  whose  clinical  symptoms  acting  particularly  upon  the  nervous 
system,  as  in  certain  forms  of  the  idiocies  just  considered,  anomalies  of  "in- 
ternal secretion"  of  different  glands,  play  the  most  important  pathological 
role,  and  which  on  this  account  I  hence  bring  together  as  "'dysglandular." 

As  is  known,  specific  substances  which  are  secreted  by  the  definite  glandular 
structures  into  the  blood  represent  an  important  physiological  factor  of  our 
organism  in  which  they  have  to  stimulate  in  a  chemical  way,  certain  functions. 
From  op/idto=  I  stimulate,  is  derived  the  name  "hormone"  proposed  by  Starling 
for  such  substances.  A  particular  affinity  of  a  great  number  of  hormones  for 
the  nervous  system  or  certain  parts  of  it,  is  just  as  important  from  a  patho- 
genetic point  of  view,  as  the  fact  that  the  disturbance  of  function  of  a  gland 
with  internal  secretion  (they  are  also  called  "endocrine  glands")  can  involve 
also  one  or  more  of  the  other  glands,  so  that  it  is  sometimes  difficult  to  decide 
certainly  as  to  which  organ  was  primarily  affected.  I  will  confine  myself 
chiefly  to  the  discussion  of  such  disease  conditions  in  which  the  nature  of  the 
endocrine  disturbances  are  relatively  plain  and  generally  recognized,  namely! 
1,  Basedow's  disease;  2,  myxoedema, — both  of  thyreogenic  origin;  3,  Addison's 
diseas< — a  consequence  of  disease  of  the  adrenals,  and,  4,  different  syndromes 
connected  with  disturbed  function  of  the  hypophysis.  The  future  will  show 
bow  many  of  the  dvskinetic  conditions  with  which  we  have  already  occupied 
ourselves  can  definitely  be  arrayed  among  the  dysglandular  affections.  For 
tetany,  as  we  have  seen,  this  is  very  probable,  but  also  in  relation  to  paroxys- 
mal paralysis,  paralysis  agitans,  etc.,  the  view  that  they  are  dependent  upon 
disturbance  in  the  action  of  hormones  is  coming  more  and  more  to  the  front. 

I.     Basedow's  Disease 

Tin's  affection  was  comprehended  in  its  clinical  individuality,  and  described 
simultaneously  (about  1K40)  by  tin'  English  clinician  Graves,  and  the  Rlerser- 
burg  physician  Basedow.  There  are.  however,  exact  descriptions  of  a  few  casts 

from  an  earlier  time,  among  which  those  of  the  Italian  Flujiini  (1802)  deserve 
Bpecial    mention.      "Morbo   di   Flajani"   IS  also   a   name    for   the  disease   used    ill 

Italy,  while  the  English  designation  is  "Graves'  disease."  Besides  "maladie  de 
Basedow,"  the  disease  i-  also  culled  in  French  after  two  of  its  most  striking 
symptoms,  "goitre  exophtalmique,"  while  the  German  term,  "Glotzaugen- 
krankheit"  has  become  almost  obsoleti  . 

.-5 15 


3iG 


LECTURE    XXIII 


Symptomatology 

The  fully  developed  cases  of  Basedow's  disease  are  characterized  by  the 
unmistakable  and  characteristic  combination  of  four  cardinal  symptoms  about 
which -again  are  grouped  a  number  of  less  striking  phenomena.  Recognizing 
that  opposed  to  these  classical  cases  there  are  a  great  number  of  "Formes 
frustes"  with  rudimentary  symptomatology,  we  will  begin  with  the  considera- 
tion of  these  cardinal  symptoms  (goiter,  exophthalmus,  tachycardia,  tremor). 

1.  The  Goiter. — This  is  in  general  not  an  excessive  struma  formation,  but 
only  a  moderate  hypertrophy  to  about  double  the  size  of  the  healthy  thyroid. 
Usually  at  once  perceptible  upon  near  inspection  of  the  neck,  it  is  often  only 

plain  upon  palpation.  Either  both 
lobes  are  equally  enlarged,  or  the  in- 
crease in  volume  is  especially  of  one 
lobe,  remarkably  more  frequent  on  the 
right.  The  goiter  is  usually  soft  and  as 
auscultation  for  murmurs  shows,  is  very 
vascular.  Not  rarely  an  arterial  thrill 
may  be  detected  upon  palpation,  and 
besides  this,  a  definite  pulsation  of  the 
gland.  This  richness  in  vessels  well  ex- 
plains the  great  variations  in  the  size 
of  the  struma,  which  can  sometimes  be 
noted  when  it  is  measured  regularly. 
For  the  rest,  it  swells  also  upon  exer- 
tion, excitement,  etc.,  sometimes  quite 
plainly.  Within  the  soft  tissue  a  few 
more  resistant  portions  are  to  be  felt 
in  some  places.  In  general  the  Basedow 
goiter,  mainly  in  consequence  of  its  gen- 
eral softness,  produces  much  fewer  sub- 
jective symptoms  than  an  ordinary 
struma  of  the  same  size.  Pressure 
symptoms  are  usually  absent  entirely; 
asphyxic  difficulties  proper,  from  com- 
pression of  the  trachea  or  the  recurrent  nerves  only  very  rarely  occur,  while 
a  feeling  of  fullness  and  tension  in  the  region  of  the  thyroid  gland  is  somewhat 
more  frequently  complained  of. 

2.  The  Exophthalmus. — The  protruding  eyes  ("Glotzauge")  of  Basedow 
patients  when  typically  developed,  give  an  uncommonlv  characteristic  expres- 
sion of  countenance;  this  has  been  called  the  "tragic  look,"  and  as  a  fact,  the 
physiognomy  in  advanced  cases  reminds  one  of  certain  masks  of  ancient  trage- 
dies (see  Fig.  100).  In  general,  however,  I  should  characterize  the  expression 
which  the  exophthalmus  gives  to  the  features  of  Basedow'  patients  rather  as 
that  of  anger,  to  which,  also,  the  swollen  neck  contributes  its  part  (see  Fig. 
101).  In  less  intense  development,  the  exophthalmus,  produces  rather  the  im- 
pression of  a  glistening  eye   ("Glanzauge")    than  of  a  protruding  eye,  while 


Fig.  100. 
"Tragic  Look"  in  Basedow's  Disease. 


DYSGLANDTLAR    SYMPTOM-COMPLEXES 


347 


on  the  other  hand,  particularly  marked  protrusion  of  the  eyeball  can  cause 
inability  to  close  the  lid  and  has  even  been  responsible  for  luxation  of  the 
eye  from  its  socket.  The  exophthalmus  is  usually  symmetrically  developed, 
though  in  a  considerable  proportion  of  cases  there  is  a  distinct  difference 
between  right  and  left.  That  the  intensity  of  the  phenomenon  shows  great 
variation  in  the  course  of  the  disease  is  quite  usual,  so  that  even  a  marked 
exophthalmus  can  disappear  again.  Three  phenomena  are  to  be  emphasized 
as  characteristic  accompanying  symptoms  of  the  protruding  eyes  in  Basedow's 
disease:  1.  "Stellwag's  sign": 
winking  is  abnormally  infrequent 
in  such  patients  and  the  palpe- 
bral fissure  is  unusually  wide,  so 
that  below  and  above  the  cornea 
si  more  or  less  wide  strip  of  the 
sclerotic  is  visible.  2.  Graefe's 
sign:  in  looking  down  a  disturb- 
ance in  the  physiological  syner- 
gy between  the  movements  of  the 
lid  and  those  of  the  eyeball  is 
apparent,  so  that  the  upper  lid 
lags  behind  instead  of  sinking 
synchronously  with  the  sagittal 
axis  of  the  eye.  3.  Mobiiis* 
sign:  the  movement  of  con- 
vergence of  the  eyes  occurs  im- 
perfectly or  is  quickly  impaired. 
This  last-mentioned  phenomenon 
is   less  constant   than    the  symp- 

t s    of    Stellwag    and    Graefe. 

3.  The  Tachycardia. — The 
pulse  is  permanently  quickened, 
so  that  even  when  lying  quietly 
on  lb,-  back  figures  of  100.  120, 
and     even      100     beats     to     the 

minute  are  observed.  Changes  of  position,  exertion  of  any  sort,  but  particu- 
larly psychical  excitement,  increase  the  pulse  frequency  excessively,  so  thai 
e\in  200  beats  a  minute  may  occur  (usually  with  a  feeling  of  severe  palpita 
tion  of  the  heart).  The  rhythm  of  the  pulse,  however,  remains  nearly  always 
"  gular.  Cardio-vascular  symptoms  accompanying  this  permanent  tachycardia 
are  quite  frequent  and  manifold.  I  would  mention  above  everything  the  in- 
creased vasomotor  irritability  of  the  integument  which,  among  other  things, 
Manifests  itself  in  so-called  dermographism:  stroking  the  skin  with  a  blunt  ob- 
ject produces,  after  a  few  seconds,  wide  vn\  stripes  in  which  the  drawing  or 
writing  produced  in  this  manner  only  pales  and  disappears  after  several  hours. 
Often  the  dermographism  is  combined  with  a  serous  infiltration  of  the  skin 
which  leads  to  the  production  of  wheals:  we  speak  then  of  factitious  urticaria. 
Congestion  of  blood  about   the  bead,  temporary  redness  which  can  alternate 


348  LECTURE    XXIII 

with  marked  pallor,  intense  feeling  of  heat  in  extended  regions  of  the  surface 
of  the  body,  are  further  phenomena  belonging  here.  The  blood  pressure  I 
have  sometimes  found  abnormally  high;  for  example,  from  140  to  185  (meas- 
ured with  Gartner's  tonometer  on  the  patient's  sitting  quiet),  and  abnormally 
labile ;  still,  these  conditions  vary  from  day  to  day  within  wide  limits.  Over 
the  heart  systolic  murmurs  are  sometimes  to  be  heard,  namely  with  increased 
tachycardia.  Their  maximum  intensity  is  usually  at  the  base;  these  are  (as 
the  inconstancy  and  not  rarely  quick  disappearance  of  these  phenomena  show), 
as  a  rule,  functional  murmurs  due  to  relaxation  of  the  atrioventricular  orifices 
and  a  resulting  insufficiency  of  the  mitral  and  tricuspid  valves ;  the  character 
of  these  murmurs  is  soft  and  blowing.  Also,  by  percussion  and  orthodiagraphy 
a  certain,  usually  inconsiderable,  degree  of  dilatation  of  the  heart  can  be  demon- 
strated in  some  cases. 

4.  The  Tremor. — This  is  an  exceedingly  rapid  and  fine  tremor  (8  to  10 
oscillations  a  second),  which  is  to  be  perceived  in  the  outstretched  fingers  of 
the  patient,  but  which  we  can  often  plainly  appreciate  when  we  place  a  hand 
upon  the  head  or  upon  the  shoulder  of  the  standing  or  sitting  patient.  Upon 
movement,  intensity  of  this  tremoi  usually  increases,  while  it  may  cease  upon 
complete  rest. 

When  now  the  four  cardinal  symptoms  are  present,  along  with  which,  be- 
sides, a  number  of  the  ocular  and  cardio-vasomotor  accompanying  phenomena 
already  mentioned  are  always  to  be  found,  we  speak,  as  already  said,  of  the 
classical  form  of  the  disease.  Frequently,  however,  we  meet  with  '"formes 
frustes"  in  which  either  the  exophthalmus  or  the  enlargement  of  the  thyroid, 
or  even  both,  are  absent,  and  only  the  heart  and  vascular  anomalies  and  the 
tremor  can  be  found.  If,  now,  for  a  diagnosis  of  Basedow's  disease,  the 
presence  of  these  two  "obligatory"  cardinal  symptoms  would  suffice,  they  must 
present,  of  course,  those  clinical  peculiarities  which  I  have  pointed  out  to  you. 
In  spite  of  this,  however,  the  inclusion  of  rudimentary  cases  of  this  kind  under 
exophthalmic  goiter  is  not  justified  if  they  do  not  present  besides  some  of  the 
rather  rich  array  of  the  so-called  "secondary  Basedow  symptoms"  with  which 
we  will  now  become  acquainted. 

The  following  disease  manifestations  of  subjective  and  objective  nature 
play,  along  with  the  cardinal  symptoms  in  the  clinical  picture  of  Basedow's 
disease,  a  role  varying  from  case  to  case  and  presenting  manifold  combinations, 
but   yet  quite   important : 

a.  Muscular  Weakness  in  the  lower  extremities,  often  in  the  form  of  a 
peculiar,  paroxysmal  paraparesis,  so  that  the  patient's  legs,  while  walking  or 
standing,  suddenly  give  way  at  the  knees.  Rarely,  however,  does  this  weakness 
increase  to  a  degree  causing  the  patient  to  be  bedridden,  and  is  even  then  tem- 
porary, since  this  symptom  in  particular  is  subject  to  great  variations  during 
the  course  of  the  disease.  Infrequently  do  these  transitory  pareses  assume  an- 
other localization,  for  instance,  hemiplegic  or  monoplegic,  or  affect  the  neck 
muscles  or  the  regions  innervated  from  the  brain.  In  the  last  case  there  may  be 
not  only  temporary  pareses,  but,  as  Stellwag  has  shown,  actual  paralyses  of' 
the  external  eye  muscles,  while  the  muscles  of  accommodation  and  of  the  iris 
are  always  spared  (except  as  an  extreme  rarity).     Here  belongs  also  the  im- 


DYSGLANDULAR    SYMPTOM-COMPLEXES  349 

possibility  of  drawing  deep  inspirations  sometimes  observed  ("Louise  Bryson's 

sign").' 

b.  Disturbances  of  Menstruation. — Dysmenorrhcea  is  very  frequent;  ainen- 
prrhoea  not  exactly  rare. 

c.  Diarrhoeas. — Charcot  and  Mobius  have  pointed  out  the  great  tendency 
of  Basedow  patients  to  profuse  diarrhoeas  occurring  in  attacks  sometimes  last- 
ing for  days  and  weeks,  which  begin  without  any  apparent  reason,  defy  opium, 
bismuth,  tannin,  etc.,  and  then  suddenly  and  for  no  apparent  reason,  cease. 
The  evacuations  (up  to  10  and  more  per  day)  are  very  thin  and  often  entirely 
painless. 

d.  Hyperidrosis. — Excessive  sweat  secretion  may  he  general  or  it  may  be 
limited  to  a  definite  portion  of  the  body  (face,  hands,  feet):  it  may  be  con- 
tinuous, or  there  may  be  profuse  outbreaks  of  sweat  occurring  in  attacks 
usually  coincident  with  congestions  and  palpitation  of  the  heart.  Also  in 
such  patients  who  do  not  complain  of  excessive  sweat,  even  when  the  tem- 
perature is  low,  the  skin  is  found  in  the  condition  for  which  I  would  borrow 
the  French  expression  "peau  moite"  (moist  skin).  Yigouroux's  phenomenon 
is  to  be  attributed  to  this  abnormal  moisture  of  the  integument:  this  is  re- 
duction of  the  electrical  resistance.  If  this  last  is  in  healthy  persons  about 
4.000  ohm-,  in  Basedow  patients  it  often  sinks  to  1,000  ohms  and  below.  To 
obtain  a  certain  number  of  milleamperes  in  galvanization,  we  need  in  these 
pases  to  switch  in  only  one-quarter  as  many  elements  as  for  a  normal  person, 
corresponding  to  Ohm's  law: 

_  .   _,,  ,,  Electromotive  Force 

Current  strength  =  —      .  , 

Resistance 

c.  Psychic  Changes. — Most  Basedow  patients  show  a  marked  hasty  and 
restless  manner,  a  '"feverish  motor  impulsion,"  which,  however,  is  accompanied 
bv  great  defecfin  staying  power  when  the  task  is  once  begun.  They  find  it 
difficult  to  remain  long  seated  or  standing  in  the  same  place;  their  mood  is 
extremely  labile:  their  expressions  of  joyful,  of  sad  emotions  are  boundless, 
and  in  conversation  they  are  wordy,  precipitate  and  inclined  to  deviation  from 
the  subject  under  discussion.  Their  surrounders  often  complain  of  the  ex- 
aggerated sensitiveness,  the  irascibility,  the  capriciousness  of  such  patients. 
Like  the  other  disease  symptoms  these  psychic  symptoms  are  subject  to  the 
i  variations  in  their  intensity,  can  disappear  in  the  course  of  the  affec-" 
tion  and  reappear.  .More  severe  psychic  disturbances,  psychoses  proper,  de- 
velop in  some,  fortunately  rare  cases.  Homburger  has  furnished  proof  thai 
a  specific  "Basedow  insanity,"  as  was  asserted  by  former  authors,  does  not 
exist.  Rather  doc  s  Basedow's  disease  occur  in  connection  with  almosl  all 
forms  of  insanity,  a  coincidence'  which  probably  is  to  be  explained  through 
the  st, mi  t  lines  severe  iieuro-ps vchopat hie  heredity  of  Basedow  patients, 

/.  Neuralgic  Phenomena.  Eye  neuralgias,  intercostal  neuralgias,  pain  in 
the  trigeminus  distribution,  particularly  in  the  upper  branches,  are  the  most 
frequent  manifestations  of  these  troubles,  not  rare  in  Basedow  patients.  The 
pains  are.  as  a   rule,  of  only  moderate   intensity;   paresthesias   may   occur   also, 

g.  Signs  of  over  irritability  in  the  neuro-muscular  apparatus.  Light  tap 
bing  of  the  muscles  usually  calls   forth  a  definite  contraction,  and  by   firmer 


350  LECTURE    XXIII 

striking,  one  can  bring  out  sometimes  the  phenomenon  of  "idio-museular  con- 
traction" with  which  we  became  acquainted  when  speaking  of  tetany.  On  the 
other  hand  the  appearance  of  either  mechanical  or  electric  overirritability  on 
percussion  or  on  galvanic  stimulation  of  the  nerve  trunks  is  very  rare,  while 
only  as  a  very  unusual  occurrence  has  the  appearance  of  tetanoid  spasms  been 
described.  As  to  the  "choreiform  movements"  to  which,  among  others,  Ray- 
mond and  Dieidafoy  have  alluded,  I  believe  that  they  may  have  been  only  a 
specially  striking  degree  of  intensity,  of  the  already  mentioned  psycho-motor 
restlessness  of  Basedow  patients.  In  a  great  majority  of  cases  you  will  find 
the  tendon  reflexes,  very  often  also  the  skin  reflexes,  abnormally  lively. 

h.  Trophic  Disturbances. — Falling  out  of  the  hair,  combined  with  dryness, 
brittleness  and  lack  of  lustre  has  been  considered  by  Curschmann  as  a  very 
important  Basedow  symptom  and  actually  occurs  in  my  experience  in  about  60 
per  cent,  of  the  cases,  however,  usually  only  to  a  moderate  extent,  so  that 
the  patient's  attention  must  be  called  to  it.  Less  frequent  are  pigment  anom- 
alies, usually  in  the  form  <>f  a  hyper-pigmentation,  about  the  face,  neck,  ab- 
domen, etc.  (similar  but  less  intense  than  those  which  we  will  soon  meet  when 
describing  Addison  s  disease),  here  and  there,  however,  on  the  contrary,  in  the 
form  of  a  pathological  loss  of  pigment,  vitiligo.  As  rarities  may  be  men- 
tioned, also,  a  firm  but  fleeting  oedema  on  the  body  and  the  lower  extremities, 
further,  abnormal  softness  of  the  bone  and  atrophic  changes  in  the  breasts, 
while  the  observations  of  sclerodermic  phenomena  (see  below,  Lecture  XXV) 
in  Basedow  patients  (Lathe,  Stahelvn  and  others)  practically  do  not  indicate 
anything  more  than  a  combination  of  two  distinct  disease  conditions. 

i.  Anomalies  of  Metabolism. — The  investigations  of  Fr.  Mutter,  Magnus 
Levi/,  Scholz,  and  others  have  proved  that  in  Bascdozc's  disease  the  excretion 
of  nitrogen,  carbonic  acid,  phosphoric  acid  can  experience  a  pathological  in-, 
crease,  which  probably  depends  upon  increased  destruction  of  albumin  in  con- 
sequence of  an  elevation  of  the  total  metabolism.  Xo  wonder,  that,  as  a  rule, 
even  when  taking  plenty  of  nourishment  there  is  often  a  tendency  to  extreme 
and  rapid  emaciation  (in  one  case  of  Mannheim's  the  weight  fell  45  kilos,  in 
10  months).  Huchard  lias  referred  to  "crises  of  emaciation"  occurring  parox- 
ysmally.  On  the  other  hand,  there  arc  Basedow  patients  who  are  corpulent 
and  remain  so.  Polyuria,  eventually  with  polydipsia,  is  not  entirely  rare, 
also  alimentary  glycosuria,  while  true  diabetes  mellitus  has  only  been  de- 
scribed in  a  few  cases  (mostly  as  a  complication  in  the  advanced  stages). 
Rises  of  temperature  during  shorter  or  longer  periods  are  also  an  unusual 
symptom,  while  transitory  albuminuria  of  slight  degree  (without  casts)  is 
somewhat  more  frequently   found. 

A\  Blood  Changes. — As  characteristic  for  Basedozc's  disease,  Kochcr  con- 
siders the  following  hematological  finding:  actual  increase  of  the  number  of 
lymphocytes,  causing  relative  diminution  of  the  polynuclear  leucocytes,  with 
red  corpuscles  normal  in  number  and  in  hemoglobin  content. 

Course  and  Prognosis 

The  disease,  which  affects  by  preference  the  female  sex  in  the  second  and 
third  decade,  but   occurs   even   in   children,  begins   usually,  but  by   no   means 


DYSGLANDULAR    SYMPTOM-COMPLEXES  351 

always,  by  the  generally  gradual,  only  exceptionally  sudden,  appearance  of 
a  "cardinal  symptom,"  and,  indeed,  in  almost  two-thirds  of  the  cases  with  the 
heart  troubles,  more  rarely  with  the  struma  or  the  tremor,  most  rarely  with 
ocular  symptoms.  The  further  course  is  only  in  the  mildest  cases  subchronic 
(of  several  months'  duration),  in  cases  of  moderate  severity  chronic,  remittent 
and  intermittent,  extending  over  years.  Malignant  cases  with  great  intensity 
of  the  symptoms  characterize  themselves  sometimes  by  acute  or  hyperacute 
course  (in  cases  of  Trousseau  and  Fr.  M tiller,  after  about  2  months,  in  one 
case  of  Mackenzie,  in  3  days,  after  the  abrupt  beginning  of  the  first  symptom, 
death  occurred).  Usually,  however,  severe  disease  pictures  develop  through 
the  chronic,  progressive  increase  of  originally  slight  disturbances.  The  prog- 
nosis depends  in  the  first  place  upon  the  intensity  of  the  clinical  manifestations 
and  upon  the  possibility  of  appropriate  care  and  manner  of  life;  if  this  last 
condition  can  be  obtained,  even  apparently  hopeless  cases,  though  not  cured, 
may  again  be  brought  into  a  tolerable  permanent  condition.  Along  with  the 
duration  of  the  disease,  for  the  prognostic  estimation  of  the  individual  ease, 
the  condition  of  the  heart  comes  into  consideration  above  everything  else, 
since  heart  weakness,  with  its  consequences  (which  we  naturally  do  not  include 
under  the  symptoms  proper  of  Basedow's  disease),  also  anasarca,  ascites,  oedema 
of  the  lungs,  congestion  of  the  liver,  congestion  of  the  kidneys,  asystole,  form 
along  with  general  marasmus  the  chief  proximate  causes  of  death  in  the  lethally 
ending  cases.  A  specially  wretched  complication  of  the  most  severe  Basedow 
pases  may  he  mentioned  here:  the  keratitis  due  to  the  excessive  exophthalmos, 
the  inability  of  the  eyelids  to  close  and  the  drying  of  the  cornea,  which  may 
lead  to  the  perforation  of  the  eyeball  and  panophthalmitis  with  all  its  frightful 
consequences. 

Pathogenesis  and  Etiology 

How  is  this  peculiar  disease  picture,  so  rich  in  symptoms,  produced?  This 
question  (in  consequence  of  the  inconstancy  and  ambiguity  of  the  sparse  dis 
closures  which  pathological  anatomy  has  furnished  us)  it  has  been  attempted 
to   answer   in   many   different   ways. 

Many  of  these  theories  have  long  ago  fallen  into  oblivion  after  their  all 
too  apparent  conflict  with  the  data  of  actual  observation  has  been  shown: 
for  example,  it  was  suggested  that  the  symptom-complex  was  due  to  the 
compression   of  the   vessels  and   nerves  of  the   neck   by   the  goiter. 

There  have  remained  as  a  subject  of  discussion  only  those  views  supported 
upon  facts  which  either  have  asserted  a  primary  nervous  basis  for  Basedow's 
disease,  or  whieh  have  spoken  Jfor  the  thyreogenic  autotoxic  nature  of  its 
symptoms.  Today  lioth  views  have  justification;  both  are  sanctioned  by  the 
result  of  clinical  and  experimental  observation.  It  is  not  understandable  why 
it    is   attempted   to   bring  these   two   views   into  antithesis  witll   one  another. 

That  since  Charcot.  Basedow's  disease  ha-  been  included  among  nerVGUS 
diseases  has  a  good  clinical  hasis.  First,  the  hereditary  and  family  rela- 
tions   to   the   psychoses   and    psychoneuroses   which   are    recognizable    in    most 

cases;  second,   its   frequent    coexistence  with   almost    all    forms  of  psychoneuroses 

and    insanity    which    either    precede    the    onscl    of    Basedow's    disease    begin 


352  LECTURE    XXIII 

simultaneously  with  this  last,  or  develop  in  its  later  stages ;  third,  its  occur- 
rence as  a  complication  in  organic  diseases  of  the  nervous  system  (tabes,  mul- 
tiple sclerosis,  paresis)  ;  fourth,  finally,  its  beginning  in  countless  cases  in 
connection  with  a  fright  or  some  other  psychical  shock.  But  why  cannot  this 
neurosis  be  a  secretory  neurosis  of  the  thyroid?  Do  we  not  know  numerous 
cases  of  the  quantitative  influencing  of  the  most  varied  secretory  processes 
by  functional  as  well  as  organic  affections  of  the  nervous  system  (nervous 
gastroxynsis,  sialorrhoea,  colica  mucosa,  etc.)?  On  the  other  hand,  it  cannot 
be  denied  that  there  are  cases  enough  in  which  the  functional  anomaly  of  the 
thyroid  gland  appears  to  play  the  role  of  a  "primum  movens,"  the  remaining 
nervous  and  vascular  components  of  the  syndrome  that  of  secondary  symp- 
toms. These  cases  have  been  denominated  as  "Basedowified  goiter"  and  sepa- 
rated from  true  goiter.  We  can  easily  class  both  categories  together  and 
assume  that  the  anomalies  of  the  thyroid  gland  function  coming  into  question 
arise  not  exclusively  upon  the  basis  of  the  neurosis,  but  sometimes  also  upon 
that  of  the  local  affection.  Indeed,  for  both  varieties,  according  to  the  results 
of  clinical  experience  and  experimental  investigation,  the  two  following  points 
may  be  regarded  as  proven : 

1.  In  the  center  of  the  disease  picture  stands  the  altered  thyroid  gland 
function. 

2.  The  point  of  attack  of  the  toxic  action  proceeding  from  this  lies  in 
definite  portions  of  the  nervous  system. 

Recognition  of  the  first  forms  the  basis  of  the  "thyreogenous  theory"  of 
Basedow's  disease.  This  is  supported  by  a  number  of  experiences  which  I  will 
shortly  indicate.  Cure  of  Basedow's  disease  by  thyroidectomy;  appearance 
of  Basedow's  symptoms  in  inflammation  of  the  thyroid  or  carcinoma,  after 
excessive  use  of  thyroid  tablets  for  therapeutic  purposes ;  identity  of  the 
anomalies  of  metabolism  on  thyroid  feeding  with  those  of  Basedow's  disease 
(Magnus  Lei//)  ;  acute  appearance  of  Basedow  symptoms  on  the  overwhelm- 
ing of  the  organism  with  expressed  thyroid  gland  products,  as  sometimes 
occurs  in  goiter  operations,  the  so-called  "acute  post-operative  thyroidism"; 
more  or  less  extensive  analogy  of  the  experimental  hvperthyreosis  (transplan- 
tation, feeding,  injection  experiments  by  Ballet,  Enriquez,  Lanz,  and  others) 
with  the  picture  of  human  Basedow's  disease.* 

As  to  the  point  of  attack  of  the  thyreogenous  noxious  agent,  it  is  to  be 
placed  either  in  the  sympathetic  or  in  the  medulla  oblongata.  The  sympa- 
thetic theories  (Bencdiht,  Friedreich,  Etderiburg,  Abadie)  have  much  that  is 
seductive,  as  the  tachycardia,  the  vaso-dilatation,  the  ocular  symptoms,  the 
hyperidrosis,  etc.,  can  well  be  brought  into  connection  with  disease  of  the 
sympathetic,  as  you  will  soon  recognize  in  the  lecture  on  sympathetic  affec- 
tions.     If  we  do  not   find  cither  the  assumption   of  simple   irritation  or  that 

*  The  value  of  this  last  demonstration  has  experienced  a  considerable  limitation  through  the 
discovery  of  E.  Bircher,  that  after  transplantation  of  viable  thymus  into  the  abdominal  cavity 
of  dogs,  Basedow's  symptoms  also  appear.  However,  the  relations  between  Basedow's  disease 
and  the  most  varied  glands  with  internal  secretion  is  an  exceedingly  interesting,  though  still 
insufficiently  investigated  subject.  Whether  the  extirpation  of  remains  of  the  thymus  proposed 
by  Garri  will  establish  itself  in  the  treatment  of  Basedow's  disease  remains  to  be  seen. 


DYSGLAXDULAR    SYMPTOM-COMPLEXES  353 

of  paralysis  alone  satisfactory,  this  is  not  in  contradiction  with  the  experi- 
ences of  neuro-pathology,  in  which  the  most  varied  examples  of  combined 
irritative  and  defect  symptoms  in  the  same  nerve  territory  are  found.  Rather 
must  the  absence  of  pupillary  symptoms  arouse  consideration.  Anatomical 
changes  in  the  gangliated  cord  have  been  described  in  isolated  cases,  and  the 
physiological  proof  has  been  furnished  that  the  tachycardia  produced  by 
thyroid  juice  depends  upon  irritation  of  the  accelerator  nerves. 

Of  late,  however,  those  who  hold  that  the  point  of  attack  of  the  noxious 
agent  in  Basedow's  disease  is  in  the  bulb  have  become  more  and  more  numer- 
ous. More  important  than  the  heterogeneous  and  not  very  convincing  autopsy 
findings  (into  which  I  will  not  enter)  are  the  experiments  of  Filehrie,  Durdufi, 
and  Bienfait,  who  in  animals  set  up  a  part  of  the  Basedow  syndrome  by  lesions 
of  the  restiform  bodies,  and  especially  those  of  Tedeschi,  in  which  the  experi- 
ment only  succeeded  when  the  animals  were  in  possession  of  their  thyroids. 

Personally,  I  am  of  the  conviction  that  still  other  parts  of  the  nervous 
system  come  into  consideration,  since  how  can  we  otherwise  explain  the  psychic 
and    paraplegic    disturbances? 

Allow  me  to  anticipate  somewhat,  and  to  remark  that  myxoedema,  with 
which  we  will  occupy  ourselves  after  Basedow's  disease,  and  which  depends  upon 
secretory  insufficiency  of  the  thyroid  gland  (whether  on  account  of  its  opera- 
tive removal  or  its  pathological  elimination),  may  be  considered  the  exact 
clinical  antithesis  of  the  latter.  In  myxoedema  metabolism  is  reduced,  in 
Basedow  increased;  in  the  former  the  temperature  is  reduced,  in  the  latter 
increased;  the  skin  in  the  first  is  dry  and  thickened,  in  the  second  hyperidrotic 
and  thin;  on  this  account,  in  the  first  instance  there  is  elevation,  in  the  second 
reduction  of  the  electrical  resistance;  in  myxoedema  there  is  corpulence,  in 
Basedow  almost  always  emaciation;  in  the  one  there  is  small  and  slow  pulse, 
in  the  other  bounding  and  rapid  pulse;  here  mental  slowness,  there  excitation; 
in  the  former  the  movements  are  stiff  and  slow,  in  the  latter  hasty  and  tremu- 
lous, etc.  Mobius  first  expressed  the  idea  that  in  Basedow's  disease  there  is 
overproduction  of  a  secretion  which,  produced  in  normal  quantity,  is  intended 
to  neutralize  the  toxic  product  of  metabolism,  which  one  can  designate  briefly 
as  "myxedema  poison."  Also  in  Basedozc's  disease  there  is  a  pathogenic 
action  of  this  excess  of  hormone,  its  symptoms  are  of  a  hyperthyreotic  nature. 

The  substance  at  fault  is  probably  in  the  first  instance  iodothyrin,  discov- 
ered by  Banmann,  for  which  also  speaks  the  tact  that  iodin  medication  exerts 
an  unfavorable  influence  upon  Basedow's  disease;  indeed,  as  Kocher  and  others 
have  shown,  forced  iodin  cures  lead  to  a  syniptoniatologically  similar  disease 
picture,  the  so-called  "iodin-B  asedow."  Indeed,  iodothyrin  is  not  the  sole 
active  constituent  of  the  thyroid  gland,  but  still  other  substances  (organic- 
bases,  thyreoproteid,  etc.)  come  into  consideration.  Just  to  this  multiplicity 
of  active  substances,  which  in  pari  seem  to  show  antagonistic  action,  I  havi 
ascribed  the  fact  that  certain  symptoms  of  Basedow's  disease  and  of  myxoe- 
dema are  the  sam< — irregularity  of  menstruation,  pigmentation  of  the  skin, 
digestive  disturbances,   falling  out   of  hair.      Hyperthyroidism   may   well   be 

accompanied    by   dyst  hyroiclism    (faulty   composition    of   the    secretion). 

Xow  as  to  the  "formes  frustes"  without  enlargement  of  the  thyroid  gland, 


354  LECTURE    XXIII 

it  must  be  pointed  out  that  a  secreting  parenchyma  may  be  found  in  chronic 
functional  hyperactivity  without  hyperplasia.  (I  would  remind  you,  for  ex-* 
ample,  of  gastrorrhcea  acida.)  As  Oppenheim  suspects,  however,  the  obstruc- 
tion of  the  intralobular  lymph  channels — a  regular  lesion  of  the  Basedow 
thyroid  can  cause  an  inundation  of  the  venous  blood  with  the  products  of 
secretion  of  the  thyroid.  It  is,  however,  also  conceivable,  that  exceptionally 
the  sympathetic  or  bulbar  lesion-complex  characteristic  for  Basedow's  disease 
may  be  produced  by  other  factors  than  thyroidism. 

Treatment 

The  treatment  of  Basedow's  disease  in  most  cases  makes  great  demands 
upon  the  persistence  of  the  physician  and  of  the  patient.  Though  it  is  not 
rarely  permitted  to  us,  even  after  a  short  time,  to  note  decided  improvement, 
we  should  not  be  too  ready  to  fold  our  hands  and  let  the  patient  get  out  of 
sight.  Only  by  therapeutic  effort,  extending  over  months  if  not  years,  will 
it  be  permitted  to  us,  first  to  render  durable  the  improvement  obtained,  then 
to  increase  it,  and  finally,  often  enough,  to  procure  permanent  recovery. 

Of  capital  importance  in  this  connection  is  careful  regulation  of  all  the 
conditions  of  life  of  the  patient.  Except  in  very  mild  forms,  one  will  do  well 
to  begin  the  treatment  with  several  wTeeks'  rest  in  bed,  which  also,  in  the 
further  course  of  the  disease,  may  be  occasionally  introduced  with  advantage. 
The  excitability  and  the  tachycardia  are  usually  quite  plainly  influenced  by 
this  simple  measure.  As  an  ideal  regime  is  to  be  regarded,  the  ovo-lacto- 
vegetarian  diet  which  permits  the  preparation  of  quite  tasty  and  varied  bills 
of  fare ;  besides  this,  we  forbid  the  stimulating  spices. 

The  avoidance  of  irritants  which  the  spices  and  the  extracts  of  meat  prove 
themselves,  acts  not  only  sedatively  upon  the  nervous  system  in  toto,  but 
specially  plainly  upon  the  cardiac  and  vasomotor  innervation ;  also  tea,  coffee, 
alcoholics  and  tobacco,  are  best  forbidden  upon  the  same  grounds.  Some 
concessions,  especially  with  regard  to  the  use  of  meat,  can  be  made  according 
to  the  individuality  of  the  case.  Where  possible,  however,  it  should  always 
be  brought  about  that  the  extractives  are  only  taken  in  small  quantities,  in 
that,  for  example,  the  portions  of  fish,  poultry,  veal  or  beef  allowed  daily 
should  be  taken  after  the  juice  is  expressed. 

The  tendency  to  diarrhoea  is  not  to  be  too  much  considered  in  the  choice 
of  food,  since  the  character  of  the  latter  appears  to  be  of  slight  influence  upon 
the  intestinal  troubles  of  Basedow  patients.  Where  milk  is  unpleasantly 
laxative,  I  rub  up  10  grms  of  pure  powdered  gum  arable  in  some  cold  milk, 
then  add  it  to  a  14  liter  of  milk,  boil  it,  and  finally  add  1  bitter  and  1  sweet 
almond,  each  blanched  and  ground. 

Next  to  rest  in  bed,  it  is  well  to  prescribe  freely  sojourn  in  an  elevated 
region.  Besides  moderate  elevation,  2,500  to  4,000  ft.,  in  most  cases  residence 
in  the  high  valleys,  from  4.000  to  6,000  ft.,  can  be  recommended  with  advan- 
tage. The  Engadine  furnishes  all  the  degrees  of  elevation  coming  into  consid- 
eration. But  also  the  Black  Forest  and  other  wooded  hill  regions  give  satis- 
factory results,  while  residence  by  the  sea  is  usually  disadvantageous.     A  cer- 


DYSGLANDULAR    SYMPTOM-COMPLEXES  355 

1  measure  of  bodily  exercise  in  the  open  air,  increased  little  by  little,  should 
united  in  climatic  cures  with  several  hours  daily  reclining  in  the  open  air. 
>rts  proper,  as  well  as  dancing,  should  be  strictly  forbidden;  automobile 
ng  also  appears  to  me  to  act  disadvantageously ;  excitement  is  to  be  avoided 
much  as  possible,  especially  that  in  the  sexual  sphere. 

Under  hydrotherapy  there  come  into  consideration  carbonic  acid  baths, 
cool  frictions,  tepid  fan  or  rain  douches  of  short  duration,  slowly  cooled  from 
20  to  18°  C.     I  thoroughly  disapprove  of  "cold  water  cures"  proper. 

Electric  treatment  in  different  forms  has  been  applied.  In  my  experience, 
however,  good  results  are  obtained  only  from  the  stabile  galvanization  of  the 
sympathetic  in  the  neck  (anode  over  the  top  of  the  sternum,  cathode  at  the 
ungle  of  the  jaw,  the  current  is  slowly  raised  from  3  to  5  milleamperes,  5 
minutes'  application  to  each  side),  provided  that  daily  treatment  can  be  car- 
ried out   for  several  weeks  successively. 

Our  list  of  drugs  furnishes  some  agents  of  valuable  efficiency  in  the  appli- 
cation of  which  you  will  do  well  to  vary  from  time  to  time.  In  the  first  place, 
[  would  mention  sodium  phosphate,  which,  given  in  adequate  doses  (6,  8,  10 
^rm  per  day,  dissolved  in  water,  soup  or  milk),  appears  to  me  to  act  almost 
is  a  specific  on  most  .of  the  Basedow  symptoms.  Whether  it  exercises  this 
iction  through  its  property  of  being  an  antidote  to  iodin,  as  Kocher  thinks, 
>r  in  some  other  pharmacodynamic  manner,  I  will  not  attempt  to  decide,  and 
mlv  affirm  the  imperative  fact;  sodium  phosphate  must  be  given  in  relatively 
arge  quantities,  since  it  is  only  to  a  small  extent  absorbed.  More  absorbable 
md  assimilable  are  the  organic  phosphoric  combinations,  for  example,  calcium 
flycero-phosphate  (0.25  grm  4  times  daily).  I  only  give  it  preference  over 
odium  phosphate  when  this  last  sets  up  diarrhoea. 

We  give  bromin  and  valerian  preparations  symptomatically  for  jactita- 
ion.  for  which  the  List  is  in  general  to  be  preferred,  since  besides  this  it 
s  the  most  efficient  internal  medication  which  we  have  at  our  disposal  for 
he  cardiac  and  vasomotor  disturbances  of  innervation.  The  result  is,  how- 
ver,  only  to  be  expected  with  some  certainty  when  the  valerian  preparation 
s  given  continuously  and  in  large  doses  which  I  have  denominated  "valerian 
aturation."  By  preference  I  order  valerian  tea.  It  is  most  efficient  when 
t  is  taken  according  to  the  following  directions:  In  the  morning  the  patient 
(Ids  a  heaping  tablcspoonful  of  valerian  roof  to  a  large  cup  of  cold  water 
nd  lets  it  stand  all  day,  occasionally  stirring  it  ;  before  bedtime,  then,  it  is 
iltered  through  linen,  expressed,  and  the  concentrated  infusion  so  obtained  is 
ucen.  The  next  cup  of  tea  is  immediately  prepared  in  the  same  manner,  is 
>ut  on  the  night  table,  and  taken  immediately  after  awakening.  Boiled  \alc 
ian  tea,  or  that  made  with  hot  water,  acts  much  more  weakly,  since  many  of 
he  ethereal  components  and  volatile  acids  are  thereby  driven  off.  The  wretched 
aste  of  valerian  tea  has  created  a  need  for  a  number  of  new  valerian  specialties 
Valyl,  Bornyval,  Gynoval,  etc.)  which,  given  in  proper  doses,  also  act  quite 
■'II.  but   are  rather  expensive  and  only  applicable  to  elegant   practice. 

I  would  warn  against  the  use  of  digitalis  and  strophanthus  except  in  such 
tages  of  the  disease  in  which  heart  insufficiency  and  asystole  dominate  the 
ucture.      Apart    from    the    above-mentioned    indications    in    which    they    fill    a 


356  LECTURE    XXIII 

decided  indication,  these  cardiac  remedies  accomplish  nothing  in  Basedow'^, 
disease,  if  they  do  not,  indeed,  injure.  Belladonna  preparations  and  atropin, 
which  are  adapted  to  the  symptomatic  treatment  of  the  hyperidrosis,  have  in 
certain  cases  also  a  favorable  influence  upon  the  disease  picture  as  a  whole, 
as  Gowers  and  Grasset  have  shown.  The  use  of  ergot  preparations  and 
quinine  appear  to  me  to  rest  more  upon  theoretical  grounds — attempting  to 
act  upon  the  vessels — than  upon  favorable  and  empirical  results.  Arsenic 
medication,  on  the  contrary,  properly  enjoys  the  greatest  popularity  in  the 
treatment  of  Basedow's  disease;  beginning  with  small  doses  as  customary. 
we  gradually  increase  to  larger  ones ;  for  instance,  acid,  arseniosi,  0.002  tc 
0.005  (gr.  1/30  to  gr.  1/12)  twice  a  day,  best  in  pill  form,  as  Asiatic  pill 
or  sol.  Fowleri,  3  to  7  drops  t.i.d.,  or  sodium  cacodylate,  daily  0.05  to  Q.\l 
(gr.  %  to  gr.  2)  subcutaneously.  Also  courses  of  Levico-,  Roncegno-,  Va 
Sinestra  or  Durkheimer  arsenic  waters  are  in  place. 

Most  interesting  are  the  attempts  at  opotherapy  based  upon  the  thyroge 
nous  theory  of  Basedow's  disease.  So  it  has  been  attempted  to  introduce  int< 
the  patient  the  antagonistic  "myxoedema  poison."  Already,  in  1895,  Bala 
and  Enriquez  had  injected  the  serum  of  dogs  in  whom  the  thyroid  had  beei 
removed,  with  encouraging  results;  only  since  1901,  however,  lias  a  serun 
preparation  more  simple  in  application  the  "antithyreodin"  of  Mobius-MerM 
which  is  obtained  from  the  serum  of  sheep  deprived  of  their  thyroids  and  ordere. 
in  doses  of  from  1.5  to  6.0  a  day,  has  come  into  commerce.  In  practice  th 
use  of  the  flesh  of  thyroidectomized  animals  (Sorgo)  and  the  serum  of  myxce 
dema  patients  (Burghardt-Blumenthal)  have  fallen  entirely  out  of  use,  stil 
more  so  the  anthropophagous  methods  of  Lanz  and  Mobius  (the  admimstra 
tion  of  thyroid  substance  from  cretins).  On  the  other  hand,  the  milk  o 
thyroidectomized  goats  (introduced  into  commerce  in  a  pulverized  conditio 
as  "Rodagen")  has  come  into  use  after  the  advice  of  Lanz.  Finally,  in  Franc 
the  glycerinized  blood  of  thyroidless  animals  has  been  given  as  "Hemato-ethj 
roidine." 

Most  recently  it  has  been  endeavored  to  prepare  a  serum  containing  th 
specific  cytolysines  directed  against  the  thyroid  gland;  the  interesting  result 
of  various  experimenters  (Marikowslcy,  MacCallum,  Lepine,  and  others)  ha\ 
not,  however,  proved  of  practical  value. 

The   pathogenetically    so    rational    opotherapeutic   measures   have   not   | 
become  established  as  a  part  of  the  therapy  of  Basedow's  disease,  and  it 
questionable  if  this  will  ever  be  the  case.     According  to   their  nature,  thj 
substances  exert  only  a  temporary  action,  and  are  able  to  influence  the  sevei 
cases,  which,  as  has  been  recognized,  are  insusceptible  to  recovery— only  du 
ing  the  time  that  they  are  being  administered,  and  here,  even,  on  account  < 
the  high  price  of  these  anti-thyroid  remedies  (a  course  of  antithyreoidin  ma 
cost   five   hundred   francs),   they   can   rarely  be   chosen.      Their   indication 
found  above  everything  in  acute  intermediate  cases  where  there  is  danger 
delay,   for  example,   from   attacks   of   suffocation.      A  protracted  use   of  tl 
antithyroid  remedies  will  only  take  precedence  over  the  other  methods  alreac 
mentioned  in.  those  fortunately  rare  cases  of  very  severe  Basedow's  disease. 

A  formidable  rival  to  them,  besides  the  of  late  recommended  X-ray  trea 


DYSGLAXDULAR    SYMPTOM-COMPLEXES  357 

ment  of  goiter,  has  been  developed  in  surgical  treatment.     With  a  description 
of  this  last  we  will  close  this  lecture. 

Practically  abandoned  is  resection  of  the  sympathetic  in  the  neck  which 
Jaboulai/  attempted  to  establish.  This  operation  can,  indeed,  favorably  in- 
fluence goiter,  exophthalmus  and  tachycardia,  but  only  temporarily,  and  has 
also  acted  unfavorably.  The  same  author  has  recommended  "exothyropexia," 
in  which  the  thyroid  is  drawn  out  through  an  incision,  covered  with  a  dressing, 
and  left  to  the  spontaneous  atrophy  which  occurs  in  favorable  cases ;  this 
operation  also  has  been  abandoned  (on  account  of  the  dangerous  results  which 
have  repeatedly  followed  it).  Tli.  Kocher  favors  partial  strumectomy  in  com- 
bination with  ligature  of  the  arteries,  and  with  him  the  majority  of  surgeons 
agree.  One  must  not  conceive,  however,  that  the  results  which  this  operation 
often  produces  in  an  astonishingly  short  time  must  be  permanent,  and  only 
too  often  one  sees  rapid  return  of  the  troubles  in  their  former,  or  even  in 
greater  intensity,  which  has  often  led  to  a  new  partial  resection  of  the  thyroid. 
The  mortality  of  thyroidectomy  in  Basedow  patients  is  about  5  per  cent., 
mainly  on  account  of  the  vascularity  of  the  goiter  and  the  labile  condition 
of  heart  and  vasomotor  nerves.  It  will  be  well  to  reserve  operative  inter- 
ference for  severe  cases  which  do  not  react  to  other  methods,  but  here,  how- 
ever, not  to  put  off  this  last  resort  until  the  disease  has  reached  a  stage  at 
which  life  is  directly  threatened.  Although  there  are  surgeons  who  on  prin- 
ciple advise  against  any  strumectomv  in  Basedow's  disease  (for  example,  the 
well-known  student  of  goiter,  Heinrich  Bircher),  on  the  other  hand,  even  in 
some  cases,  which  from  their  nature  could  be  very  well  cured  bloodlessly,  in 
practice,  on  economic  and  social  grounds  we  cannot  oppose  operation,  since 
these  patients  on  the  one  hand  are  doomed  to  invalidism  through  the  severity 
of  their  trouble,  on  the  other  are  not  in  a  position  to  afford  a  long  rest  cure, 
change  of  climate,  etc. 


LECTURE  XXIV 
Dysglandular  Symptom-Complexes 

II.     Myxoedema 


The  pathological  condition  to  whose  description  we  must  now  proceed  was 
first  studied  clinically  by  the  Englishman  Gull,  in  the  year  1873,  but  received 
its  present  name  5  years  later  from  Ord,  since  which  time  the  name  proposed 
by  Charcot,  "cachexie  pachydermique,"  has  become  almost  entirely  obsolete. 
The  most  important  step  in  the  recognition  of  this  disease  was  furnished  by 
the  discovery  by  the  Geneva  surgeons,  J.  and  A.  Reverdin,  that  after  total 
extirpations  of  the  thyroid  gland,  a  condition  entirely  analogous  to  spon- 
taneous myxoedema  appeared  ("cachexia  strumi-priva,"  after  Kocher).  On 
this  account  the  conception  that  spontaneous  myxoedema  also  is  a  hypo-  or 
athyreosis,  a  more  or  less  complete  insufficiency  of  the  internal  secretion  of 
the  thyroid  gland  imposes  itself.  Eicald  has  found  pathologico-anatomical 
support  for  this  view:  He  showed  that  the  thyroid  in  almost  all  cases  of 
myxoedema  which  had  come  to  autopsy  was  atrophic,  cirrhotic,  showed  a  de- 
struction of  the  parenchyma  of  the  gland,  with  fibrous  proliferation  of  the 
interstitial  tissue,  and  also  that  in  the  macroscopically  enlarged  thyroid  glands 
of  myxoedema  patients,  the  increase  in  volume  is  caused  by  connective  tissue 
increase  at  the  cost  of  the  secreting  epithelium.  It  is  noteworthy  that  some- 
times enlargement  of  the  hypophysis  has  been  found  in  myxoedematous  con- 
ditions (perhaps  to  be  considered  as  an  attempt  of  the  organism  to  effect 
compensation). 

The  chief  symptoms  of  myxoedema  are  a  peculiar  alteration  of  the  integu- 
ment— bv  infiltration  of  the  meshes  of  its  connective  tissue  with  a  mucoid 
semi-fluid  mass — and  intellectual  disintegration.  Along  with  this  there  is 
also,  in  congenital  myxoedema  ( thyreoaplasia)  and  infantile  myxoedema,  re- 
tardation of  the  growth  of  the  skeleton;  since,  however,  we  have  already 
become  acquainted  with  both  these  forms  as  sporadic  cretinism,  we  will  con- 
sider to-day  only  the  myxoedema  of  adults,  and  first  in  its  spontaneously 
appearing  form. 


SPONTANEOUS    MYXOEDEMA    OF    ADULTS 


As  exciting  causes  of  this  form,  like  in  its  pathogenetic  antithesis,  Base- 
dow's disease,  psychic  traumata  (fright,  excitement,  etc.)  appear  to  play  a 
certain  role.  Also  infectious  diseases  and  loss  of  blood  (particularly  in  conse- 
quence of  repeated  difficult   labors)    have  been  accused.     Certain  relations  to 

358 


DYSGLANDULAR    SYMPTOM-COMPLEXES  359 

the  function  of  the  female  genital  organs  are  made  probable  in  that  women 
are  -i  times  as  frequently  affected  as  men;  virgins,  however,  very  rarely. 
Also,  a  local  predisposition  without  doubt  exists.  In  England  and  France, 
for  example,  spontaneous  myxoedema  occurs  more  frequently  than  in  Ger- 
many, Austria  and  Switzerland  (although  the  two  last  countries  show  numer- 
ous endemics  of  goiter  and  cretinism).  In  England  hereditary-family  occur- 
rence also  does  not  appear  to  be  very  infrequent. 

A  rapid  development  of  the  symptoms  is  entirely  unusual,  rather  do  these 
generally  establish  themselves  gradually  and  little  noticed  in  the  course  of 
years,  until  in  typical  cases  the  following  exceedingly  characteristic  disease 
picture  is  present:  The  skin  takes  on  a  pale  yellowish  coloration  and  an 
(Edematous  appearance,  though  pressure  of  the  finger  does  not  leave  behind 
the  pitting  so  characteristic  of  renal  and  cardiac  anasarca;  this  is  a  hard 
and  elastic  infiltration,  a  pachydermia;  the  face  upon  whose  waxlike  pallor 
two  rose-red  spots  appear  over  the  cheeks,  is  swollen  like  a  full  moon;  the 
thickened,  deformed  eyelids  make  the  palpebral  fissure  appear  very  narrow ; 
the  clucks  appear  puffed  out,  the  lips  form  thick  swellings,  and  are  slightly 
cyanotic  in  color;  the  forehead  lies  in  coarse,  immovable  folds,  the  physiognomy 
is  expressionless,  often  dull.  The  fingers  are  like  blocks,  sausage-shaped,  tin 
wide,  plump  hands  remind  one  of  fencing  gloves;  the  feet  are  deformed  like 
paws.  The  infiltration  of  the  skin  can  render  the  use  of  the  extremities  quite 
difficult.  The  mucous  membranes  undergo  similar  alterations  to  those  in  the 
skin ;  on  this  account  the  volume  of  the  tongue  increases  considerably  and  the 
nryxcedematous  alteration  of  the  laryngeal  mucous  membrane  makes  the  voice 
hoarse,  deep  and  monotonous.  The  sweat  secretion  ceases  entirely,  or  at 
least  becomes  markedly  limited;  the  integument  often  becomes  cracked  and 
falls  off  in  scales.  Its  electrical  resistance  is  considerably  increased,  its  sensi- 
bility dulled.  The  hair  of  the  head  and  body,  the  eyebrows  and  lashes,  fall 
out  or  become  very  sparse;  often,  also,  the  finger  nails  and  toe  nails  become 
brittle  and  are  lost.  The  movements  grow  continually  slower  and  weaker, 
although  no  paralysis  occurs.  Locomotion  is  carried  out  at  a  snail's  pace. 
Nearly  always  (there  are  exceptions)  mental  impoverishment  goes  hand  in 
hand  with  this  physical  decay.  The  patients  lose  every  interest,  become  weak 
in  memory  and  judgment.  If  one  talks  with  them,  they  must  think  a  long 
time  for  an  answer,  and  make  the  impression  that  they  are  continually  going 
to   sleep   (luring   the   conversation;   in    fact,    they   show   abnormal    sleepiness, 

torpor.  They  complain  8  ureal  deal  of  filling  cold;  the  temperature  of  the 
skin  and  the  centra]  temperature  an'  lowered,  the  las!  sometimes  below  36  < '. 
(96. H     V.).     Headaches  and  tinnitus  aurium  occur  in  a  portion  of  the  cases, 

sometimes  there  is  also  a  hemorrhagic  diathesis  (metrorrhagias,  bleeding  I'r 

the  gums,  etc.)  ;  further,  occasional  accompaniments  of  the  disease  are:  chronic 
synovitis  of  the  knee-joint,  albuminuria  with  hyaline  easts,  and  certain  blood 
alterations,  which  Vaquez  has  studied;  numerical  decrease  of  the  red  blood 
corpuscles,  with  increase  of  their  size  and  appearance  of  nucleated  erythro- 
cytes   (infantile   peculiarities   of   the   morphological    blood    picture). 

The    sexual    sphere    is    almost    always    affected;    besides    iiieiiorrlia^ia .    amen 

orrhoea  is  also  observed,  libido  disappears,  frigidity  appears;  in  men   then    Ie 


3(50 


LECTURE    XXIV 


also  impotence.  The  heart  action  is  usually  weak,  the  pulse  small,  occasion- 
ally irregular;  the  blood  pressure  is  often  lowered.  Particularly  interesting, 
since  they  stand  in  direct  contradiction  to  those  of  Basedow's  disease,  are  the 
anomalies  of  metabolism  in  myxedema  patients,  studied  by  Magnus-Levy;  gas 
interchange  is  reduced  to  about  half  the  normal,  bodily  weight  decreases  con- 
siderably; the  appetite  is  sometimes  reduced,  but  even  where  this  is  not  the 

case  a  decided  repugnance  to  meat  is  usually 
apparent.  Palpation  of  the  thyroid  gland 
is  made  mort  difficult  by  the  infiltration  of 
the  integument ;  usually  no  glandular  body 
is  to  be  felt ;  sometimes,  however,  an  ab- 
normallv  hard  one  which  is  then,  as  a  rule, 
very  small,  rarely  enlarged.  The  tendon 
reflexes  are  sometimes  normal,  sometimes 
weakened. 

If  the  disease  is  uninfluenced  therapeuti- 
cally j  it  is  slowly  progressive,  though  often 
with  remissions,  which  have  been  observed 
under  the  influence  of  summer  temperature, 
sometimes  also  upon  the  occurrence  of  preg- 
nancy. The  patients  usually  die  of  inter- 
current diseases  (particularly  phthisis), 
more  rarely  from  the  severe  cachexia,  which 
the  terminal  stage  of  mvxeedema  presents. 
As  in  Basedow's  disease,  the  "Formes 
■frustes"  of  this  disease  also  demand  our 
special  interest,  since  on  the  one  hand  they 
appear  much  less  rarely  than  the  severe 
form,  and  on  the  other  they  can  be  much 
more  easily  overlooked.  They  have  been 
designated  as  "benign  hypo-thyroidism." 
Sometimes  they  represent,  like  the  "formes 
frustes"  in  Basedow's  disease,  the  prelimi- 
nary stage  of  the  typical  symptom-com- 
plex. The  skin  change  can  be  entirely  ab- 
sent, or  may  be  present  only  as  a  suggestion 
in  the  form  of  a  slight,  nonoedematous 
swilling  of  the  face.  There  is  a  marked 
feeling  of  general  weakness  and  relaxation 
in  spite  of  very  good  appetite  and  digestive 
function.  The  urine  often  contains  some  albumin  and  a  few  hyaline  casts,  so 
that  one  might  think  of  a  chronic  rudimentary  uremia.  In  the  psychical  pic- 
ture, in  only  a  part  of  the  cases  a  certain  apathy  and  loss  of  interest  makes 
itself  apparent;  more  frequently,  however,  so  great  a  sopor  comes  over  the 
patient  upon  all  occasions  that  he  has  the  greatest  trouble  to  keep  awake,  and 
usually,  just  on  account  of  this  trouble,  seeks  professional  aid.  In  part  of  the 
cases  there  is  falling  out  of  the  hair,  which  is  often  limited  to  the  lateral  parts  of 


DYSGLANDULAR    SYMPTOM-COMPLEXES  361 

the  eyebrows.     Sometimes  there  is  a  feeling  of  cold,  weak  pulse,  sexual  indiffer- 
ence.    The  thyroid  gland  is  either  not  palpable,  or  very  small  and  hard. 

OPERATIVE    MYXEDEMA,    CACHEXIA    STRUMIPRIVA 

This  form  of  myxoedema,  since  the  serious  results  of  total  thyroidectomy 
have  been  recognized  and  this  procedure  has  been  carefully  avoided  in  goiter 
patients  (this  would  be  in  any  case  in  malignant  goiters),  is  scarcely  ever 
observed  any  more.  Still,  as  K^clier  has  shown,  it  can  arise  exceptionally, 
also  after  partial  thyroidectomy,  in  consequence  of  a  subsequent  atrophy  of 
the  portion  of  the  gland  parenchyma  left  behind.  On  the  other  hand,  cases 
of  total  strumectomy  have  been  seen  which  remained  free  from  myxoedema 
or  acquired  only  a  "forme  fruste";  they  possess,  evidently,  which  is  not  so 
rarely  the  case,  accessory  thyroid  glands.  Post-operative  myxoedema  usually 
develops  3  to  -A  months  after  the  extirpation,  in  a  somewhat  more  rapid  manner 
than  the  spontaneous  variety.  The  first  symptoms  are  feeling  of  weakness, 
shivering,  then  the  skin  becomes  altered  in  typical  manner,  the  movements  are 
slow,  the  hair  falls  out,  intelligence  decreases;  in  short,  the  clinical  picture 
corresponds  completely  with  that  of  the  spontaneous  eases.  Formerly  the 
combination  with  tetany  was  frequent;  the  importance  of  avoiding  not  only  the 
parenchyma  of  the  thyroid  glands,  but  also  that  of  the  parathyroids,  in 
goiter  operations,  was  not  yet  known.  The  younger  the  individual  the  more 
severe  the  cachexia  strumipriva;  in  children  it  leads  to  the  picture  of  sporadic 
cretinism  with  complete  idiocy  and  dwarfism. 

Treatment  of  Myxoedema 

There  is  only  one,  but  fortunately  a  quite  efficient,  remedy  for  myxoedema 
of  every  variety;  the  thyroid  gland  substance  of  animals,  which  can  be  re- 
placed by  certain  substances  isolated  from  it  (iodothyrin,  thyreoglobulin), 
but  without  therapeutic  gain  and  with  the  disadvantage  of  increased  cost. 
Without  doubt,  the  fresh  thyroid  substance  from  sheep,  cattle  or  hogs  works 
most  actively,  in  doses  of  1.5  to  3.0  gnu  per  day,  which  doses,  however,  are 
only  reached  by  degrees  (beginning  with  0.5  grin).  One  lobe  of  the  thyroid 
gland  of  a  sheep  corresponds  to  1.0  to  1.5  gnu.  The  thyroid  must  he  eaten 
raw  ("a  la  tartare,"  spread  on  bread);  however,  the  patient  soon  resists  this 
method  of  administration,  and  il  is  often  accompanied  by  difficulties  in  ob- 
taining the  very  easily  decomposable  "land  always  fresh.  On  this  account 
the  compressed  tablets  of  dried  thyroid  substance  presenl  Hie  most  convenienl 
and  useful  manner  of  administration.  If  they  are  nol  prepared  from  entirely 
fresh  material  under  aseptic  precautions,  they  can  ad  injuriously  on  account 
of  their  content  id'  ptomaines;  hence,  one  should  restricl  himself  to  tablet,  of 
known  efficacy  (as,  for  example,  Hie  "Tabloids  of  Burroughs,  Wellcome  \  Co.," 
or  Knoll's  "Thyradine") .  Both  these  products  contain  0.3  grm  glandular 
substance  per  tablet.  The  daily  dose  is  raised  slowly  and  cautiously  from 
1    to  5  nr  even   1 0  tabli  I  s   a   day. 

The  dosage  given  here  is  for  adults;  thai  for  chitdri  m  page  34  I     ! 

of  sporadic  cretinism. 


362  LECTURE    XXIV 

While  using  this  remedy,  continued  control  of  the  patient  is  necessary, 
since  under  no  circumstances  should  the  symptoms  of  intoxication  known  as 
"thyroidism"  be  allowed  to  occur;  these  manifest  themselves  in  tachycardia, 
palpitation,  jactitation,  rapid  loss  of  weight,  reduction  in  strength,  attacks 
of  vertigo,  vomiting,  diarrhoeas  and  exanthems.  The  patient  is  best  protected 
from  these  unpleasant,  often  dangerous,  incidents  by  discontinuous  adminis- 
tration ;  5  to  6  days  organo-therapy,  4  days  pause,  etc.  Decided  improve- 
ments are  obtained,  often  cures,  which  verge  on  the  marvelous.  The  infiltra- 
tion of  the  integument  gives  way  to  normal  conditions,  the  temperature  rises, 
the  hair  grows  again,  movability,  activity  of  mind  return,  etc.  If  normal 
conditions  have  been  reattained,  a  prophylactic  permanent  treatment  with 
small  doses  of  thyroid  must  be  ordered.  On  the  average  it  is  correct  to  give 
one  lobe  of  sheep's  thyroid  or  3  to  5  tabloids  or  thyraden  tablets  a  week. 
The  best  criterion  for  determining  the  efficient  dose  is,  as  Combe  has  shown, 
the  body  temperature,  which  should  remain  permanently  between  361/2  and 
37^  °  C.  As  a  regime,  this  author  recommends  chiefly  lacto-vegetarian  diet. 
Meat  is  best  permitted  only  with  the  juice  squeezed  out,  without  the  bouillon. 

III.     Adrenal  Insufficiency  and  Addison's  Disease 

Our  knowledge  of  the  functions  of  the  adrenals  (glandular  suprarenales), 
whose  role  in  the  organism  has  long  been  enveloped  in  obscurity,  has  been 
much  advanced  during  the  last  few  years  by  many  experimental  investigations. 
Indeed,  it  has  been  known  since  1855-56  that  the  adrenals  are  absolutely  neces- 
sary for  life.  This  was  proven  by  the  pathologist  Addison  in  agreement  with 
the  physiologist  Broicn-Si'quard.  To-day,  however,  we  know  many  new  and 
interesting  details  as  to  the  functions  of  these  peculiar  glands.  Among  other 
things  that  their  seci-etion  acts  as  an  antitoxic  both  against  exogenous  poisons 
(as  for  example,  certain  alkaloids),  and  also  against  the  toxines  produced 
by  muscular  exertion,  and  that  they  produce  lecithin  and  pigments  in  their 
cortical  layer,  in  their  medullary  substance,  on  the  other  hand,  adrenaline  and 
angiotonic  substance,  which  slows  and  strengthens  the  heart  beat,  causes  the 
circular  muscles  of  the  blood  vessels  to  contract,  and  decidedly  raises  the 
blood  pressure.  Through  its  special  ability  to  produce  adrenaline,  however, 
the  medullary  substance  of  the  adrenal  shows  that  it  belongs  to  the  "chromaf- 
fin system"  (called  after  its  histo-chemical  relations).  To  this  last  we  also 
assign  besides  this  a  number  of  small  structures,  the  paraganglia,  which  lie 
in  the  retroperitoneal  tissue  near  the  abdominal  aorta,  further,  diffused  cells 
within  the  sympathetic  nerves  and  ganglia,  and.  finally,  the  so-called  "carotid 
gland"  in  the  neck.  The  medullary  substance  of  the  adrenal  and  the  rest  of 
the  chromaffine  system  show  close  anatomical  and  physiological  relations  to 
the  sympathetic  nervous  system  and  have,  indeed,  been  denominated  directly 
as  the  "accessory  apparatus  of  the  sympathetic."  On  the  other  hand,  there 
appears  to  be  reciprocal  action  between  the  adrenals  (cortex  and  medullary 
substance)  and  the  chromaffine  system  on  the  one  hand,  and  the  thymus,  the 
lymphatic  apparatus  and  the  sexual  glands  on  the  other;  for  this  speak  among 
other  things,  the  hypoplasia  of  the  paraganglia  and  the  suprarenal  medullary 


DYSGLAXDULAR    SYMrTOM-COMPLEXES  363 

substance  which  is  found  in  status  thymicolymphaticus,  and  the  hypertrophy 
of  the  whole  lymphatic  apparatus  which  is  found  in  adrenal  tuberculosis; 
further,  the  occurrence  of  marked  hyperplasia  of  the  adrenal  cortex  ("struma 
suprarenalis")  in  pseudo-hermaphroditism,  in  inversion  of  the  sexual  charac- 
ters, in  precocious  puberty,  etc.  For  the  neurologist,  however,  very  special 
interest  is  presented  by  the  exceedingly  frequent  coincidence  of  defective  de- 
velopment of  the  adrenals  with  anencephaly,  microcephaly,  encephalocele,  hemi- 
oephaly  and  other  congenital  brain  defects,  a  correlation  whose  nature  is  still 
disputed  but  which  is  none  the  less  striking;  possibly  the  function  of  the 
suprarenal  gland  as  the  locality  of  production  for  the  lecithin,  necessary  for 
the  upbuilding  of  the  brain,  plays  a  role  here. 

All  these  things  have  mainly  theoretic  interest.  Clinical  importance  is  to 
be  attached  above  everything  to  the  symptom-complexes  arising  from  insuffi- 
ciency of  the  adrenal  function,  the  most  striking  of  which  is  that  known  to  us 
since  1885  as  "Addison's  disease."  About  90  per  cent,  of  the  cases  of  this 
disease  show  on  autopsy  destructive  lesions  of  the  adrenals  (usually  this  is 
tuberculosis,  more  rarely  malignant  tumors,  syphilis,  interstitial  inflammatory 
atrophies).  In  such  cases,  however,  in  which,  after  Addison's  disease,  the 
suprarenal  glands  have  been  found  intact,  their  function  has  been  plainly  sus- 
pended by  disease  of  the  chromaffine  elements  of  the  paraganglia  and  of  the 
sympathetic  plexus  of  the  abdominal  cavity  so  closely  related  to  them  physi- 
ologically. Von  Neusser  and  Bittorf,  indeed,  take  the  not  yet  generally  ac- 
cepted position  that  an  affection  of  the  splanchnic  nerves,  which,  as  Biedl  has 
shown,  contain  the  secretory  nerves  for  the  adrenals,  can  cause  Addison's 
disease.  On  the  other  hand,  the  occasional  cases  of  tuberculous  destruction 
of  the  suprarenal  glands  without  "Addison's"  are  brought  nearer  an  explana- 
tion by  an  observation  of  Wiesel,  who  in  a  case  of  this  character  found  vicari- 
ous hypertrophy  of  the  paraganglia  and  other  portions  of  the  chromaffine 
Bystem. 

Addison's  disease  begins  gradually;  it  may  be  in  a  manifestly  tuberculous, 
or  again  in  a  previously  healthy  individual;  usually  abdominal  pains  or  rapid 
loss  of  strength  arc  the  first  symptoms;  sometimes,  however,  the  peculiar 
coloration  of  the  skin,  which  Addison  denominated  "bronzed  skin,**  and  which 
also  bears  the  name  of  "melanodermia,"  occurs  at  the  start. 

At  first  appear  brown-black  spots  on  such  regions  as  are  normally  rich 
in  pigment,  or  are  Usually  expo     d   to  the  sun;   for  example,  the  groins,  about. 

the  genitals,  the  axilla,  about  the  nipples,  on  the  face,  the  neck,  the  backs  ol 

the   hand-.      These   spots    become    colli  iuua II y   darker,    and    are    finally    confluent, 

so  that  the  patients  assume  a  mulatto-like  appearance;  as,  however,  in  mu- 
latto., in  Addison's  disease,  the  matrix  of  the  nails,  as  well  as  the  palms  and 
soles,  usually  remain  free  from  pigment.  On  the  face  sometimes,  upon  a  dark 
ground,  still  darker  points  are  found,  also  the  hair  of  the  head  and  the  beard 
can  occasionally  become  darker.  Very  frequently  the  melanodermia  extends 
also  to  the  mucous  membranes,  that  is,  to  the  inner  surface  of  the  cheeks  and 
the  palate;  here,  however,  the  single  dark  spots  do  not   usually  run  together. 

The    second    cardinal    symptom    of    Addison's    disease    is    adynamia,    an    ex 

haustion  ocean-ring  Hi'""  the  slightest  use  of  the  muscles.     The  patients  on  this 


864  LECTURE    XXIV 

account  become  continually  less  able  to  get  about ;  they  assume  a  "drooping 
attitude,"  finally  become  bedridden.  With  this,  examination  of  the  function 
of  the  muscles  shows  that  there  never  is  true  paresis  or  paralysis. 

There  is  always,  too,  a  decided  relaxation  and  loss  of  energy  in  the  mental 
sphere.  All  intellectual  exertion  is  avoided  or  fails,  the  patients  become  in- 
creasingly apathetic;  the  duration  and  depth  of  sleep  are  increased.  Only 
rarely  psychic  irritative  symptoms  appear;  for  example,  deliria,  hallucina- 
tions, delusions.  As  an  anatomical  basis  of  these  anomalies,  a  diffuse  enceph-/^  * 
a  lit  is  in  the  frontal  region  has  been  accused  by  Klippel  (cncephalopathia\^ 
Addisonia). 

Sensory  disturbances  are  present  in  only  part  of  the  cases.  Usually  these 
:ui'  pains  which  are  located  in  the  epigastrium,  in  the  loins,  in  the  hypochondria, 
in  the  joints,  in  the  head,  and  sometimes  show  an  irritative,  radiating,  neuralgi- 
form character.  More  rarely,  there  is  general  hyperesthesia  of  the  integument, 
never  anesthesia  or  hypesthesia.  The  reflexes  usually  show  no  anomalies. 
Without  exception  there  are  gastro-intestinal  disturbances,  great  loss  of  appe- 
tite (rarely  and  only  episodically,  excessive  hunger),  frequent  vomiting,  con- 
stipation, in  the  later  stages  diarrhoea.  The  pulse  is  small  and  weak.  In  women 
the  menses  usually  stop.  The  blood  pressure  falls  off  decidedly ;  in  the  ad- 
vanced stages  the  body  temperature  also  (in  this,  subjective  feeling  of  cold). 
There  occur  attacks  of  vertigo,  tinnitus  aurium,  temporary  amblyopia,  syn- 
cope. The  patients  become  continually  more  and  more  anemic  and  thin,  finally 
cachectic.  In  the  preponderant  majority  of  cases,  Addison's  disease  ends 
fatally,  though  its  course  is  usually  rather  protracted  and  may  extend  over 
from  2  to  4,  sometimes  even  over  10  or  more  years.  Epileptiform  convulsions 
with  subsequent  coma  may  precede  death.  Acute  cases  in  which  exitus  takes 
place  after  a  few  weeks  or  months,  are  quite  rare;  still  rarer,  cases  terminating 
in  recovery,  which  is  chiefly  to  be  expected  in  adrenal  syphilis.  On  the  other 
hand,  extended  remissions  occasionally  occur  in  the  course  of  the  disease. 

The  most  striking  symptom  of  Addison's  disease,  the  bronzed  skin,  greatly 
facilitates  the  diagnosis  of  this  variety  of  adrenal  insufficiency  (one  should  in- 
deed, never  forget  to  exclude  other  diseases  in  which  also  a  melanodermia  may 
develop:  pigmentary  syphilides,  melanodermia  from  pediculosis  ("morbus  vaga- 
bundus")  malarial  cachexia,  cirrhosis  of  the  liver  with  pigmentation,  bronze 
diabetes,  pellagra,  Basedow's  disease,  chronic  silver  and  arsenic  intoxication. 
There  is  also  an  adrenal  insufficiency  ("hypocpinephria")  without  pigment 
anomaly,  which  most  probably  depends  upon  the  fact,  that  in  such  cases  the 
cortical  layer  is  more  or  less  spared  by  the  destructive  lesion  (Bittorf).  The 
symptomatology  agrees  otherwise  so  closely  with  that  of  Addison's  disease 
that  these  rather  rare  observations  may  be  considered  as  "formes  frustes" 
of  this. 

As  the  opposite  of  adrenal  insufficiency  may  be  considered  certain  nervous 
symptom-complexes  which,  for  example,  occurring  after  hemorrhages  into  the 
adrenal  (Materna  and  others)  have  been  included  under  the  name  "Hyper- 
epinephria"  and  considered  as  the  result  of  an  oversaturation  of  the  organism 
with  the  hormones  of  the  suprarenal  glands.  These  observations  are  still  too 
controverted  to  be  described  here. 


;:: 


DYSGLAXDULAR    SYMPTOM-COMPLEXES  365 

It  still  remains  for  us  to  describe  the  treatment  of  adrenal  insufficiency. 
Great  hopes  have  been  placed  upon  the  use  of  extracts  or  organs,  hopes  which 
have  proved  to  be  much  exaggerated,  however.  Both  the  chopped  up  fresh 
substance  of  the  suprarenal  glands  of  the  sheep  (2  to  5  grm  a  day)  and  dry 
adrenal  extract  (about  1  grm  a  day)  have  been  administered;  further,  sub- 
cutaneous injections  of  adrenalin  (1  ce  of  adrenalin  solution  (1  to  1000)  in 
250  cc  physiological  salt  solution)  have  been  tried,  in  this,  on  account  of  the 
vessel  narrowing  effect,  the  absorption  is  very  slow.  In  many,  but  not  in  all 
ciMs,  these  remedies  have  effected  definite,  though  temporary,  remissions;  a 
comparison  with  the  stupendous  results  of  thyroid  medication  in  myxoedema  is, 
however,  not  in  the  slightest  degree  possible.  Adrenalin  has,  indeed,  repeatedly 
harmed  Addison  patients.  Hence,  one  must  satisfy  himself  with  increas- 
ing as  far  as  possible  the  nutritive  value  of  the  diet  with  the  avoidance  of 
all  overexertion,  and  with  the  administration  of  tonic  remedies  (iron,  quinine, 
arsenic).  Considering  the  antitoxic  function  of  the  adrenals,  their  insufficiency 
demands  the  strictest  avoidance  of  alcohol  and  tobacco.  Constipation  should 
be  combated  with  medicines  only  with  the  greatest  caution,  since  otherwise  the 
outbreak  of  uncontrollable  diarrhoea  is  risked.  Syphilitic  patients  are  to  be 
treated  specifically;  in  adrenal  tuberculosis  Strumpell  recommends  a  cautious 
trial  of  Koch's  tuberculin. 

IV.     Acromegaly 

In  the  year  1885  Pierre  Marie  called  attention  to  a  disease  whose  chief 
svmptom  he  defined  in  the  following  manner:  "A  singular  non-congenital 
hypertrophy  of  the  extremities,  superior,  inferior,  and  cephalic,"  and  to  which 
he  applied  the  appropriate  name  of  "acromegaly."  As  a  constant  finding  in 
the  autopsies  on  such  cases,  disease  of  the  hypophysis  cerebri,  the  pituitary 
body,  was  found. 

The  disease  begins,  as  a  rule,  between  the  age  of  25  and  30  years  and  de- 
velops verv  slowly.  The  anomalies  of  growth  accompanying  it  are  often 
noticed  indirectly  by  the  patients  from  the  fact  that  from  year  to  year  larger 
hats,  gloves,  thimbles,  rings  and  shoes  must  be  used.  Also  those  surrounding 
the  patient  become  aware  of  a  gradual  increase  in  volume  of  his  nose,  his  chin, 
the  distal  portions  of  his  limbs,  and  finally  the  picture  becomes  so  characteristic 
that   any  experienced   person   can   make  the  diagnosis   at    the   first   glance. 

In  the  fully  developed  disease,  the  acromegalic  presents  coarse  features 
with  prominent  brows  and  cheek-bones,  great,  prominent  lower  jaw,  hang- 
inn-  lower  lip,  thick,  knobby,  nose ;  the  external  occipital  prqtuberance  is  ix- 
cessively  prominent  and  sometimes  the  ears  lake  part  in  the  hypertrophy. 
The  tongue  is  enlarged,  while  a  thickening  of  the  mucous  membrane  <>f  the 
palate,  the  tonsils  and  uvula  as  well  as  enlargemenl  of  the  teeth,  an-  rarities. 
Arm   and    forearm,   thigh   and    leg   are    normal:   SO   much    the   more   striking  are 

the  disproportioned  paws  in  which  the  extremities  terminate.  Usually  this 
hypertrophy  of  the  hands  and  feet  extends  chiefly  in  a  transverse,  only  rarely 
in  an  axial  direction  ("type  en  large,"  "type  en  long").     The  fingers  and  toes 

arc-  wide,  quadrangular,  the  nails  on  the  other  hand,  which  have  not  taken  part 


366  LECTURE    XXIV 

in  the  increase  of  volume,  appear  small.  The  hypertrophy  affects  both  the 
skeleton  and  the  soft  parts  (the  skin,  subcutaneous  tissues,  muscles).  Less 
constantly  than  the  limbs,  is  the  thorax  altered;  in  this  case  it  increases  par- 
ticularly in  antero-posterior  diameter,  so  that  a  kyphosis  in  the  upper  dorsal 
region  along  with  knob-like  bulging  of  the  sternum  forward  occurs;  also  the 
collar-bones  become  very  prominent.  The  neck  appears  shortened,  the  head 
drawn  down  between  the  shoulders.  The  larynx  is  often  abnormally  enlarged, 
the  voice  is  rough  and  deep,  which  is  particularly  striking  in  women.  The  skin 
is  discolored,  dry,  often  covered  with  warts,  feels  spongy  on  the  hands  and 
feet.  Falling  out  of  hair  is  rare.  Of  other  anomalies  there  have  been  found 
occasionally,  hypertrophies  of  the  viscera,  heart,  liver,  spleen,  etc.;  almost 
regularly,  on  the  other  hand,  disturbances  in  the  sexual  organs;  in  women  there 
is  early  amenorrhea  and  sterility  with  atrophy  of  the  breasts;  in  men  impo- 
tence, sometimes  also,  atrophy  of  the  genitals.  In  about  one-third  of  the  cases 
there  is  glycosuria  with  polyuria,  polydipsia,  polyphagia,  sometimes  with  the 
criteria  of  diabetes  mellitus.  Abnormal  fatigability  and  great  feeling  of  weak- 
ness, are  almost  always  present ;  sometimes  there  are  neuralgic  pains  in  differ- 
ent groups  of  muscles  which  has  been  designated  the  "hyper-algesic  form"  of 
acromegaly.  Psychically  the  patient  is  apathetic  and  permanently  out  of  sorts. 
The  tendon  reflexes  are,  as  a  rule,  normal,  sometimes  reduced. 

Acromegaly  always  has  as  its  basis,  disease  of  the  hypophysis  and  indeed, 
as  w7e  know  to-day  (thanks  to  the  most  recent  very  thorough  investigations, 
and  contrary  to  the  original  opinion  of  Marie),  in  an  alteration  leading  to 
overfunction  of  its  glandular  portion,  a  hyperpituitarism.*  These  are  chiefly 
simple  hyperplastic  tumors,  the  so-called  strumas  of  the  hypophysis,  further, 
adenomata  with  all  transition  forms  to  adenocarcinoma  and  carcinoma.  That 
tumors  of  the  connective  tissue  series  have  also  occasionally  been  found  (sar- 
coma) was  for  a  long  time  brought  forward  as  an  argument  against  the  con- 
sideration of  acromegaly  as  hyperpituitarism.  Still,  Hanau  and  Benda  have 
furnished  the  pathologico-anatomical  evidence  that  these  neoplasms  which  ap- 
pear sarcomatous  are  nevertheless  to  be  classed  among  the  malignantly  de- 
generated glandular  tissue  tumors  and  indeed,  in  4116  locations  appearing  like 
sarcoma,  are  infiltrated  with  glandular  epithelium.  Only  rarely  in  acromegaly 
is  a  hypophysis  tumor  not  present  in  such  cases,  in  part,  hypophyseal  adeno- 
mata have  been  found  in  other  locations  (for  example,  in  the  cavities  of  the 
sphenoid  bone),  in  part  there  has  been  an  increase  of  the  chromaffine  cells  of  the 
pituitary  body,  which  are  to  be  considered  as  its  functional  element,  without 
macroscopical  enlargement  of  this  latter. 

In  general,  however,  by  means  of  the  X-ray  the  hypophyseal  tumor  can 
be  recognized  intra  vitam;  the  sella  turcica  shows  abnormal  dimensions  and  is 
usually  enlarged  in  a  sagittal  direction.  Large  neoplasms  can,  besides  this, 
manifest  themselves  clinically  by  pressure  and  neighborhood  symptoms ;  severe 
headache,  cerebral  vomiting,  vertigo,  slowing  of  the  pulse,  only  very  rarely 
papilledema;  on  the  other  hand  (from  chiasma  lesions)  bitemporal  hemianopsia, 
or  even  bilateral  amaurosis.     Further,  by  paralyses  of  the  eye  muscle  nerves 

*  From  "glandula  pituitaria,"  the  old,  to-day  obsolete,  designation  of  the  hypophysis; 


DYSGLAXDULAR    SYMPTOM-COMPLEXES  3(57 

which  pass  by  the  hypophysis  on  their  way  to  the  orbit,  as  well  as  of  the  first 
branch  of  the  trigeminus. 

The  more  or  less  great  prominence  of  these  special  tumor  symptoms  is 
naturally  of  prime  importance  in  relation  to  the  course  and  prognosis  of  the 
individual  case.  Sternberg  has  differentiated  three  varieties  of  course,  perhaps 
somewhat  too  schematically;  the  usual  form,  with  a  duration  of  from  10  to 
30,  the  malignant,  with  one  of  from  3  to  i  years,  and  finally,  the  benign  form 
which  may  extend  over  even  5  decades.  In  general  the  disease  proceeds  but 
very  slowly  and  often  remains  a  long  time,  sometimes  even  definitely,  stationary. 
It  does  not  seem  susceptible  of  recovery.  The  proximal  causes  of  death  are 
intercurrent  diseases,  brain  pressure,  diabetes,  cachexia. 

Therapeutically  we  are  rather  helpless.  Extirpation  of  the  hypophysis  by 
the  nasal  or  temporal  routes  (r.  Eiselberg,  Cashing,  and  others)  is  so  exces- 
sively dangerous  and  offers  so  little  hope  of  radical  removal  that  it  must  be 
reserved. for  cases  with  marked  symptoms  of  brain  pressure,  unbearable  head- 
ache, threatened  blindness.  As  to  the  X-ray  treatment  of  acromegaly  (acting 
upon  the  hypophysis  from  the  naso-pharynx)  no  decisive  opinion  can  vet  he 
formulated.  On  the  other  hand,  repeated  courses  of  arsenic,  raising  it  to  large 
daily  doses,  appears  to  favorably  influence  the  course  in  most  cases  (Brissatid). 
The  neuralgiform  pains  and  the  diabetes  demand  symptomatic  and  dietetic 
treatment. 

In  differential  diagnostic  relations,  as  opposed  to  acromegaly,  there  come 
before  everything  else  into  consideration,  the  different  varieties  of  giantism 
produced  by  a  delay  of  the  ossification  in  the  region  of  the  epiphyseal  svn- 
fcfaondroses,  in  consequence  of  which  growth  comes  to  a  standstill  not  at  all, 
or  only  very  late,  and  Hie  skeleton  assumes  excessive  dimensions.  There  are 
distinguished  partial  giantism,  which  usually  affects  only  one  or  two  extremi- 
ties (or  indeed,  only  parts  of  them;  for  example,  in  one  case  of  Wieland's,  Only 
the  anterior  half  of  one  foot),  and  general  giantism.  The  last  stands  patho- 
genetically  plainly  wry  mar  to  acromegaly,  in  a  great  number  of  cases;  in 
mis,  indeed,  enlargement  of  the  hypophysis  cerebri  has  been  confirmed  both 
Roentgenological! y  and  on  autopsy.  If  hyperpituitarism  occurs  in  children, 
it  also  leads,  as  Brissaud  has  shown,  to  giantism,  if  it  arises,  however,  after 
ossification  of  the  epiphyseal  lines  of  the  skeleton,  it  makes  itself  evident  by 
acromegalic  changes.  In  hypophyseal  giantism,  the  genitals  usually  remain 
rudimentary,  also  the  secondary  sexual  characteristics  (heard,  puhes.  etc.). 
do  not  develop.  With  acromegaly,  further.  Marie's  "pulmonary  osteoarthro- 
pathy" (Osteoarthropathie  hypertrophiante  pneumique")  should  not  he  con- 
Osed.  In  this  last  there  is  an  enormous  drumstick-like  thickening  of  Hie  end 
phalanges  with  marked  curvation  of  the  nails  and  ankylosis  of  the  finger  and 
toe  joints  changes  which  in  patients  with  chronic  lironehiectasies  (more  rarely 
with    other   diseases    of    the    bronchi    and    lungs)    occasionally    develop. 

OTHER    DYSGLANDULAR    SYNDROMES 

If  we  now  should  go  over  all  the  dysglandular  symptom-complexes  no!  ye\ 
described,  we  would  exceed  the  limits  of  a  neurological  text  hook   in  ;l  manner 


368  LECTURE    XXIV 

which  would  be  as  little  justified  by  the  slight  occurrence  of  nervous  disease 
pictures  in  agenitalism  and  hypogenitalism  (castrated  and  eunuchoid  persons), 
in  infantilism  and  its  different  varieties,  and  in  the  "pluriglandular  insuffi- 
ciency" resulting  from  the  secretory  disturbances  of  numerous  glandular  ap- 
paratus, as  by  the  slight  practical  importance  of  these  conditions.  Hence  we 
will  content  ourselves  with  a  short  consideration  of  two  clinical  pictures  which 
indeed  are  very  rare,  but  as  the  newest  'acquisitions  to  the  subject  which  has 
occupied  us  for  the  last  two  lectures,  attract  actual  interest. 

Adiposo  Genital   Degeneration    (Frohlich's   Disease, 
Hypophyseal  Eunuchism) 

This  rare  disease  is  due  to  hypophyseal  tumors  which  cause  no  hyper- 
function  of  the  pituitary  gland,  but  rather  act  destructively,  also  to  traumatic 
lesions,  as,  for  example,  the  entrance  of  a  rifle  bullet  into  the  sella  turcica  in 
an  observation  of  Modelling.  It  is  hence  a  clinical  expression  of  hypopitui- 
tarism, of  hypophyseal  insufficiency  which  is  confirmed  by  the  improvement  of 
thr  symptoms  upon  the  administration  of  hypophyseal  substance.  Sometimes 
disease  processes  which  are  located,  not  in  the  hypophysis  itself,  but  in 
other  parts  of  the  base  of  the  skull,  by  "neighborhood  action"  can  lead  to 
the  cardinal  symptoms  of  adiposal  genital  degeneration :  excessive  develop- 
ment of  fat,  along  with  arrest  of  development  of  the  genitals  and  of  the  second- 
ary sexual  characteristics  (respectively  disturbance  of  function  and  atrophy 
of  the  sexual  organs  in  case  the  disease  occurs  after  puberty).  Sometimes  this 
hypophyseal  eunuchism  is  combined  with  dwarfism.  That  the  genital  disturb- 
ance is  common  to  hyper-  and  hypopituitarism,  may  appear  paradoxical ;  still, 
Basedow's  disease  and  myxcedema  have  traits  in  common,  for  example,  dis- 
turbances of  digestion,  irregularity  of  the  menses,  falling  out  of  the  hair. 

Dyspinealism 

Only  most  recently  has  it  been  made  known  through  Marburg  in  Vienna 
that  the  epiphysis,  the  pineal  gland,  is  a  blood  gland,  whose  hormone  during 
childhood  has  important  influence  upon  the  mental  and  physical  development 
of  the  individual.  On  this  account,  in  children,  certain  tumors  in  the  region 
of  the  corpora  quadrigemina,  along  with  the  correlates  of  disturbances  directed 
into  neighboring  neurones  (paralyses  of  the  pupil  and  the  external  eye  mus- 
cles, ataxia,  hardness  of  hearing,  visual  disturbances)  produce  the  following 
symptom-complex:  Hyperplasia  of  the  genitals,  abnormal  growth  of  the  body 
in  length,  unusual  growth  of  hair,  sexual  and  mental  precocity.  Marburg  as- 
sumes in  these  cases  an  insufficiency  of  the  pineal  secretion ;  the  last  acts,  in 
that  it  normally  delays  the  sexual  development,  antagonistic  to  the  hypophy- 
seal secretion. 


LECTURE  XXV 

Diseases  of  the  Sympathetic,  Angio-  and  Tropho- 

Neuroses 

Gentlemen:  That  portion  of  our  nervous  system  of  whose  disturbances 
we  will  speak  to-day,  lias  other  attributes  than  the  brain,  spinal  cord,  motor, 
sensible  and  sensory  nerves.  Separated  from  our  consciousness  and  our  will, 
to  a  certain  extent  independent  of  the  cerebrospinal  apparatus,  it  appears  not 
by  far  to  reach  the  importance  of  the  latter.  Indeed,  it  plays — in  the  lan- 
guage of  A.  v.  Kolliker — while  the  brain  like  a  mighty  ruler  is  enthroned  high 
up  in  the  state  chambers  of  the  skull,  only  the  role  of  a  servant,  who  exercises 
in  the  lower  rooms  of  the  body  his  appointed  functions.  And  still,  this  is  not 
without  importance,  and  the  brain  itself  is  in  the  last  instance  dependent  on  it. 
Indeed,  for  the  whole  life  of  the  individual  as  well  as  for  that  of  the  race,  a 
system  which  regulates  the  heart's  action,  the  whole  circulation,  the  secretion 
and  the  processes  of  nutrition  as  well  as  reproduction,  has  a  fundamental  im- 
portance. 

This  nervous  system  has,  as  the  sympathetic  or  autonomic,  been  contrasted 
with  the  cerebrospinal,  as  the  visceral  with  the  somatic  and  as  the  vegetative 
with  the  animal  nervous  systems.  Of  late,  however,  it  has  become  customary  no 
longer  to  use  promiscuously  the  words  "sympathetic"  and  '•autonomous,"  but 
to  apply  the  term  "sympathetic"  to  the  system  of  the  gangliated  cords,  and  to 
reserve  the  word  "autonomous"'  for  the  visceral  nervous  apparatus  in  the  head 
and  the  pelvic  organs  (cranial  and  sacral-autonomous  systems).  A  separation 
lulu  these  two  subvarities  is  justified  among  other  things  by  differences  in 
their  susceptibility  to  toxicological  influences  which  have  become  known  to  us, 
particularly  through  the  brilliant  work  of  the  English  physiologist  Langley, 
to  discuss  which  at  length,  however,  would  lead  us  too  far  afield. 

We  cannot,  however,  neglect  to  sketch  briefly  the  principles  which  control 
tin-  anatomical  structure  and  the  physiological  differentiation  of  the  visceral 
nervous  system.  Its  peculiar  eriterion  as  compared  to  the  somatic  nervous 
system  is  the  fact  that  numerous  nerve  cells  are  interpolated  in  its  course  far 
into  the  periphery.  This  interpolation  occurs  in  two  ways  in  that,  on  the  one 
hand  sympathetic  cells  are  grouped  in  compact  ganglia  which,  connected  with 
'■in  another  by  sets  of  fibers,  form  t  he  so-called  "gangliated  cord";  on  t  he  other 
hand,  however,  both  in  the  neighborhood  of  the  viscera  and  also  in  the  substance 
of  these  themselves,  still  further  collections  of  cells  are  to  he  found.  As  you 
know,  the  gangliated  cords  extend  along  in  front  of  the  vertebral  column  on 
cither  side  from  the  neck  to  the  coccyx   and  are  in  communication   through 

369 


370 


LECTURE    XXV 


the  "rami  communicantes  albi"  (white  communicating  brandies)  with  the  spinal 
roots  and  the  ganglion  cells  of  the  spinal  cord ;  on  the  other  hand,  they  give 
off  the  "rami  communicantes  grisei"  (gray  communicating  branches)  which 
enter  the  spinal  nerves  and  intimately  mixing  with  their  fibers  pass  farther, 
finally  to  reach  the  blood  vessels,  and  to  undertake  their  innervation.  The 
sympathetic  ganglia  not  belonging  to  the  gangliated  cord  (the  latter  is  called 
by  Gaskell  "the  lateral,"  the  former,  the  "collateral"  ganglion  system)  are 
connected  with  it  through  the  visceral  plexuses.     These  last  are  distinguished 


Fig.  103. 

Plan  of  the  Sympathetic  Nervous  System. 

Rm  =  Spinal  cord;  Hw=Posterior  root;  Vw=  Anterior  root ;  Spg  =  Spinal  ganglion;  Spn  = 
Spinal  nerve;  Gst  =  Gangliated  cord  (Patera!  ganglion  system);  pGg1  =  Peripheral 
ganglion  in  the  neighborhood;  pGg2  =  Peripheral  ganglion  in  the  wall  of  the  innervated 
organ  (Collateral  ganglion  system);  PI  =  Sympathetic  plexus;  0  =  Innervated  organ 
(hollow  organ). 


from  the  plexuses  of  the  somatic  nerves,  on  the  one  hand  by  their  net-like 
structure,  on  the  other  by  their  gray  color  (without  medullary  sheath).  Col- 
lateral ganglia  arc,  for  example,  the  coeliac,  solar,  mesenteric  ganglia  in  the 
abdominal  cavity,  Ludwig's,  Bidder's,  Remak's  ganglion  in  the  heart  wall.  It 
is  important  that  a  different  and  to  a  certain  extent  antagonistic,  functional 
significance  is  attributed  to  the  lateral  and  to  the  collateral  sympathetic  gan- 
glion system.  The  neurones  arising  from  the  cells  of  the  lateral  system  of 
ganglia,  that  is,  from  the  gangliated  cord,  subserve  vaso-constriction,  con- 
traction of  the  circular  muscles  of  the  hollow  organs  and  acceleration  of  the 
heart.     On  the  other  hand,  those  from  the  collateral  collections  of  cells,  effect 


SYMPATHETIC,    AXGIO-    AND    TROPHONEUROSES         371 

vasodilatation,  contraction  of  the  longitudinal  muscles  and  heart  inhibition. 
Specially  in  regard  to  this  last  it  must  be  emphasized  that  the  cardiac  plexus 
receives  an  important  addition  from  the  vagus  nerve  (which,  however,  also 
anastomoses  with  the  sympathetic  plexuses  of  the  lungs  and  stomach,  as  well  as 
with  the  cceliac  plexus)  and  we  already  learned  in  Lecture  II  that  irritation  of 
this  nerve  slows  the  movements  of  the  heart.  Is  there  in  this  an  encroachment 
of  the  cerebrospinal  system  upon  the  function  of  the  visceral?  Are  there  in 
this  way  vegetative  functions  exercised  directly  by  the  brain  without  the  inter- 
mediary' of  the  sympathetic?  Not  at  all.  The  vagus  has  in  its  role  as  a  heart 
nerve,  only  to  be  considered  in  principle  a  "ramus  communicans  albus."  The 
inhibitory  apparatus  proper  of  the  heart  are  intracardiac  ganglia — belonging 
to  the  collateral  system — which  give  off  their  "post-ganglionic"  inhibiting 
fibers  to  the  heart  muscle. 

A.     Diseases  of  the  Sympathetic 

The  pathology  of  the  sympathetic  must,  up  to  the  present,  be  designated 
as  that  department  of  nervous  diseases  in  which  there  is  the  least  actual  knowl- 
edge, and  it  has  been  attempted  to  supply  its  place  by  more  or  less  well-founded 
hypotheses.  So  of  late,  some  authors  of  the  Vienna  School  (Eppinger,  Hess 
and  others)  sketch  a  symptom-complex  which  may  owe  its  origin  to  a  hyper- 
function  of  the  vagus  or  of  the  autonomous  system,  under  the  name  of 
"vagotony."  Such  patients  manifest  narrowness  of  the  pupils  (irritation  of 
the  ciliary  ganglion),  salivation  and  lacrimation  (irritation  of  the  chorda 
tympani  and  of  the  lacrimal  nerve),  byperidrosis  (irritation  of  the  sweat 
nerves),  bradycardia,  irregular  respiratory  pulse,  respiratory  arhythmia  (irri- 
tation of  the  heart  and  lung  vagus),  bronchial  asthma  (irritation  of  the  smooth 
muscle  fibers  of  the  bronchial  tubes),  hyperacidity  and  increased  .stomach  peri- 
stalsis  (irritation  of  the  stomach  vagus),  and  still  other  symptoms. 

The  above-mentioned  authors  support  their  pathogenetic  conception  of 
these  conditions  upon  a  certain  pharmaco-dynamic  reaction  of  the  patient  in 
question:  all  the  symptoms  of  "vagotony,"  according  to  them,  disappear  after 
large  doses  of  atropin,  on  the  contrary,  undergo  considerable  exaggeration  after 
the  administration  of  pilocarpin,  while  the  so-called  adrenalin  glycosuria  is  ab- 
sent after  adrenaline  injections.  Further  investigations  must  show  in  how  far 
such  a  localization  of  a  clinical  syndrome  can  lay  claim  to  proof  by  means  of 
experimental    pharmacology. 

Best  known  and  most  simply  explainable  physio-pathologically,  is  that 
-Miiploiu  which  is  produced  by  a  lesion  of  the  sympathetic  in  the  neck  and 
which  we  call  "Ilonur'x  symptom-complex."  We  have  already  pointed  out 
( s(c  page  1  Mi )  that  through  the  third  ganglion  of  tin-  cervical  gangliated  con  I, 
the  fibers  from  the  ciliospinal  center  of  the  lowest  cervical  segments  of  the 
cord  run  to  the  eve:  they  innervate  1,  the  superior  tarsal  muscle  :  "J,  the  orbital 
muscle;  :i.  the  dilator  pupilhe;  loss  of  their  function  hence  causes  enophthal 
urns  (sinking  in  of  the  eyeball ),  narrowing  of  the  palpebral  fissure  (sympathetic 
ptosis)  and  paralytic  myosis  (see  Fig.  104).  Since  now,  however,  the  cervi- 
cal sympathetic  also  contains  vasomotor  and   sweat    secretory   fibers   lor   the 


372 


LECTURE    XXV 


corresponding  half  of  the  face,  there  results  further  from  their  elimination  a 
vasomotor  paralysis  in  this  region ;  this  manifests  itself  in  fresh  cases  by  heat 
and  reddening,  later,  however,  usually  by  cyanosis  and  coldness  (see  Lecture  I, 
page  9),  and  anidrosis  of  the  same  region.  Still,  in  Horner's  symptom-com- 
plex there  is  quite  frequently  hvperidrosis  of  one  side  of  the  face,  hence  irrita- 
tive and  defect  symptoms  seem  combined.  As  causes  of  Horner's  syndrome  the 
following  factors  acting  upon  the  cervical  sympathetic  come  into  considera- 
tion: Goiters,  wounds  (also  operative)  glandular  tumors,  for  example  malig- 
nant lymphoma,  '"cervical  ribs,"  etc.  Not  rarely,  however,  the  most  exact 
examination  does  not  permit  the  discovery  of  such  causal  factors  and  we  have 


Fig.  104. 

Horner's  Symptom-Complex. 

(Enophthalmus,  narrowing:  of  the  palpebral  fissure  myosis,  in  lesion   of  the  cervical 
sympathetic  on  the  left  side.) 


then  before  us  a  pathological  manifestation,  harmless  in  itself,  which  we,  lack- 
ing a  bitter  explanation,  must  designate  "neurotic."  A  unilateral  symptom  of 
irritation  of  the  cervical  sympathetic  occurs  relatively  frequently  in  tuber- 
culosis of  the  lung  apex,  in  that  upon  the  side  of  the  pulmonary  lesion  the 
pupil,  eventually,  also,  the  palpebral  fissure,  is  widened. 

As  "neuroses"  ami,  indeed,  as  "vasomotor-trophic  neuroses,"  are  described 
a  number  of  interesting  disturbances  etiologically  unfortunately  quite  obscure 
but  in  any  case  acting  upon  the  sympathetic  distribution,  of  which  we  will  now 
consider  a  few.     These  are  acroparesthesia,  angiospastic  dysbasia,  angiospastic 


SYMPATHETIC,    AXGIO-    AND    TROPHONEUROSES         373 

gangrene,   scleroderma,   neurotic   dropsy,   erythromelagia,   facial   hemiatrophy 
and  hemihypertrophy. 

B.      Acroparesthesia 

Acroparesthesia  (the  name  originated  with  Schultze),  readily  the  most  fre- 
quent of  the  vasomotor  neuroses,  affects  in  the  great  majority  of  cases  women, 
and  appears  most  frequently  between  the  ages  of  40  and  55  years.  The  climac- 
teric appears  to  create  a  special  predisposition,  as  does  also  general  nervosity 
(neurasthenia  or  hysteria)  ;  the  frequency  of  the  disease  in  individuals  whose 
extremities  are  particularly  exposed  to  wet  anil  cold,  for  example,  washwomen, 
points  to  the  influence  of  exogenic  factors. 

The  disease  manifests  itself  in  very  unpleasant  paresthesias,  occurring  in 
attacks,  sometimes,  indeed,  increasing  to  actual  pains,  which  are  located  mainly 
in  the  hands,  less  frequently  in  the  feet.  The  causes  of  these  unpleasant  sen- 
sations (formication,  prickling,  tickling,  stabbing,  "going  to  sleep,"  etc.)  are 
spasmodic  conditions  of  the  peripheral  arteries,  which  are  shown  by  the  livid 
coloration  resulting  from  pallor  and  cyanosis,  which  can  be  perceived  in  severe 
cases  during  the  acroparesthetic  attack,  in  the  affected  parts  (while  slight  at- 
tacks present  no  visible  alteration).  The  attacks  occur  by  preference  at  night 
or  in  the  early  morning  (when  the  blood  pressure  is  lowest),  and,  as  a  rule, 
bilaterally.  After  the  attack  passes  away,  sometimes  reactive  reddening  of 
the  fingers  or  toes  occurs.  In  the  great  majority  of  cases  there  arc,  during  the 
attack,  occasionally  also  intcrparox vsmall v,  slight  disturbances  of  sensibility 
in  the  affected  extremities;  tiny  present  their  maximum  in  the  pulps  of  the 
fingers  (or  toes)  and  rapidly  diminish  proximallv.  Dejerine,  Trombert,  and 
others,  have  occasionally  observed  along  with  this  typical  condition  ('•ischemic 
disturbance  of  sensation"  of  Schlemiger),  radicularly  arranged  hypesthesias 
in  acromegaly;  in  spite  of  many  reexaminations  I  have  not  so  far  been  able  to 
confirm  these  observations. 

The  diagnosis  of  the  disease  is  usually  exceedingly  easy,  only  one  must 
remember  that  a  number  of  other  affections  commence  with  paresthesias  of 
the  fingers  or  toes,  or  can  do  so.  So,  ergotism,  "intermit  tint  limping,"  "dys- 
basia  angiosclerotica  intermittens"  (see  Lecture  XV,  page  225),  respectively 
its  analogue  located  in  tin'  upper  extremities  ("dyspraxia  angiosclerotica  in- 
termittens"), tabes  dorsalis,  tetany,  acromegaly,  and  finally,  as  we  will  soon, 
sec,  angiospastic  gangrene  {Raynaud's  disease  which  is  in  so  far  related  to 
acroparesthesia  that  it  presents  a  malignant  increased  degree  of  intensity  of 
the  "vaso-constrictor  neurosis  of  the  extremities").  In  any  case,  also  tie  re- 
lations between  acroparesthesia  and  vasomotor  angina  pectoris  (Nothnagel, 
Curshmann)  in  which  also  by  preference  in  the  night  and  morning  hours,  the 
same  subjective  and  objective  anomalies  appear  on  the  fingers,  but  also  with 
palpitations,  precordial  anxiety,  pain  in  the  region  of  the  heart  with  radiation 
into    the    left    arm,    also    the    sympt  oms    of    Inir    angina    pectoris,    are    close.       In 

contradistinction    to   the   latter,   however,   there   are   absent    all   symptoms  of 

sclerotic   or   luetic   diseases   of   the   aorta    nr    the   coronary    vessels;    rather   does 

this   evidently   benign  disease   affect   generally,  physically  quite  healthy   per- 


374  LECTURE    XXV 

sons,  usually  hysterical  young  women.  Psychical  traumata  are  nearly  always 
discovered  in  the  etiology;  Curshmann  accuses  among  other  things  coitus  in- 
terruptus. 

The  prognosis  of  acroparesthesia  cannot  be  laid  down  generally.  There 
are  cases  which  resist  all  treatment,  though  they  usually  present  an  alternation 
of  improvements  and  relapses  (such  remissions  usually  occur  during  the  warm 
part  of  the  year).  On  the  other  hand  I  have  seen  not  a  small  number  of  cases 
pass  on  to  recovery  and  many  permanently  improve  to  an  entirely  tolerable 
stage.  As  to  life,  the  disease  is  entirely  harmless ;  as  to  function,  not  all  too 
disturbing,  since  it  scarcely  ever  causes  actual  disability. 

From  a  therapeutic  point  of  view  electrical  applications  are  to  be  recom- 
mended in  the  first  place:  The  faradic  brush,  faradic  hand  baths,  "four  cell 
baths."  Further,  a  course  of  quinine  from  time  to  time  procures  for  many 
such  patients  a  permanent  or  lengthy  improvement.  At  bedtime  a  capsule  of 
0.5  quinine  sulphate  or  "chinin-phytin"  (combination  with  phosphorus)  is 
given  every  night  for  from  2  to  4  weeks.  Between  these  quinine  cures  proper, 
quinine  in  small  doses  can  be  given  further,  very  well  in  the  form  of  the  fol- 
lowing pills : 

R^      Quinin   sulph 3.0   (grs.   45) 

Ext.    nucis    vom 1.5    (grs.  22) 

Ext.   hyoscyami    2.5   (grs.  38) 

Ext.   valerian    7.0   (grs.   105) 

M.     Fiat  Pil.  No.  C.    S.    2  pills  t.  i.  d.  after  meals. 

For  washing,  only  warm  water  must  be  used.  At  night  the  patient  should 
wear  fur-lined  slippers  or  socks. 

C.     Angiospastic  Dysbasia 

The  "intermittent  limping,"  occurring  from  organic  disease  of  the  periph- 
eral, also  of  the  spinal  vessels,  we  have  already  become  acquainted  with  in 
Lecture  XV.  There  is  now,  however,  a  purely  vasomotor  form  of  this  syn- 
drome to  which  Oppenheim  first  called  attention  and  which  of  late  has  been 
accurately  studied  by  Curschmann.  The  first  author  observed  a  case  of  in- 
termittent claudication  for  15  years,  without  the  severe  results  of  vascular 
obstruction  having  developed.  On  this  account  there  presented  itself  to  him  the 
possibility  that  permanent  spastic  conditions  of  the  muscles  of  the  artery  wall, 
though  varying  in  their  intensity,  can  occur,  and  can  produce  the  intermittent 
limping.  He  also  expressed  the  suspicion  that  a  congenital  narrowness  of  the 
vascular  system  prepared  the  way  for  the  occurrence  of  this  disturbance. 
Curschvuinn  saw  in  people  from  18  to  22  years  old  intermittent  limping  with 
all  subjective  symptoms,  also  with  the  absence  of  pulse  in  some  of  the  arteries 
of  the  foot,  and  could  certainly  exclude  arteriosclerosis  and  arthritis.  By 
plethysmographic  investigations  he  was  led  to  assume  the  possibility  of  a  per- 
manent spasmodic  condition  of  the  arteries.  I  myself  have  classed  as  vaso- 
motor neurotic  dyspraxia  of  the  upper  extremity,  forms  of  writer's  cramp  which 
were  accompanied  by   coldness   and  lividity   of  the  hand,  but  the  cases  men- 


SYMPATHETIC,    AXGIO-    AND    TROPHONEUROSES         375 

tioned  are  on  the  whole  quite  infrequent  and  I  would  impress  it  upon  you  that 
the  diagnosis  of  a  functional,  that  is,  a  benign  form  of  intermittent  limping 
or  similar  functional  disturbances  of  the  upper  extremities,  should  in  any  case 
only  be  made  after  long  and  thorough  observation,  and  in  case  of  doubt  al- 
ways to  suspect  an  organic  vascular  lesion  and  to  make  this  supposition  the 
basis  of  your  treatment. 

This  last,  however,  will  be  in  general  the  same  in  angiospastic  and  in 
angiosclerotic  intermittent  dysbasia.  The  use  of  alcohol  is  to  be  limited, 
tobacco  forbidden.  Not  less  important  is  the  absolute  prohibition  of  those 
perverted  therapeutic  measures  to  which  such  patients  only  too  often  turn  on 
their  own  account  or  from  unwise  counsel ;  cold  wate.r  procedures,  hot  foot- 
baths, excessive  gymnastics  and  massage,  forced  marches,  etc.  The  same  thing 
applies  to  the  use  of  rubber  stockings  and  bandages.  To  be  avoided  are  all 
substances  having  decided  vasomotor  action,  strong  tea,  coffee,  spices. 
Further,  care  should  be  taken  that  the  feet  and  legs  are  kept  evenly  warm 
(proper  choice  of  residence  and  work-room,  foot  and  leg  coverings,  bedclothes, 
occasionally  mild  rubbing  with  tepid  water).  Hot  foot-baths  are  to  be 
avoided;  warm  foot-baths,  on  the  other  hand,  to  be  recommended,  as  also  ap- 
plications of  Fango,  peat,  Priessnitz  compresses,  etc.  Erb  has  quite  warmly 
recommended  the  galvanic  foot-bath ;  either  in  the  fashion  that  each  foot  is 
placed  in  a  separate  tub  containing  salt  water  at  from  34  to  36°  C.  on  a 
pole  plate  and  a  stabile  current  of  from  12  to  20  milleamperes  is  conducted 
through  them,  first  in  one  direction  and  then  in  the  other  for  from  3  to  6  min- 
utes, or  so  that  both  feet  are  placed  in  a  tub  containing  the  cathode  while  the 
anode  rests  over  the  sciatic  in  the  bend  of  the  knee,  or  over  the  plexus  on  the 
sacrum. 

Of  drugs  vaso-dilator  substances  come  into  consideration  in  the  first  place 
(courses  of  quinine  as  in  acroparesthesia,  also  nitrites,  nitro-glycerin  and 
diuretin  have  been  recommended)  ;  in  the  second  place  heart  tonics,  particularly 
those  from  which  a  better  blood  supply  and  favoring  of  the  collateral  circula- 
tion are  to  be  expected,  without  action  upon  the  vasomotors;  also  particularly 
the  strophanthus  preparations  in  contradistinction  to  digitalis.  Simple  or 
carbonated  salt  hat  lis  are  to  be  recommended  on  account  of  their  vaso-dilator 
effect.  Of  great  importance,  finally,  is  the  regulation  of  the  movements  and  of 
tin-  use  of  the  legs.  At  first,  when  possible,  entire'  rest  ami  long  reclining  are 
to  he  prescribed.  In  any  event,  however,  all  violent  exertion  is  to  he  for- 
bidden and  walking  is  to  be  permitted  only  to  such  an  extent  as  does  not  pro- 
duce any  difficulty.  Only  when  decided  improvement  has  been  obtained,  the 
patient,    '"watch   in    hand,"    is    allowed    to    undertake   more    prolonged    exercise, 

D.     Angiospastic  Symmetrical  Gangrene,  Raynaud's  Disease 
("Asphyxie  Locale  Symmetrique") 

This   very    rare   disease-    I    have   only    seen    three    cases       is    to   he   considered 

as  the  highest  stage  of  intensity  of  the  vaso-constrictor  neurosis  of  the  ex- 
tremities. The  occasional,  hut  by  no  means  regularly  observed  pathologico- 
anatomieal  findings  (  endartcrit ie  ami  endophlebitic  changes)  are  readily   I"  be 


376  LECTURE    XXV 

considered  as  secondary.  Raynaud's  disease,  in  contradiction  to  acropares- 
thesia, shows  no  special  predilection  for  either  sex,  and  usually  occurs  in  the  com- 
paratively young,  at  between  25  and  35  years  of  age.  Cassircr,  indeed,  saw  a 
nursling  affected  with  symmetrical  gangrene.  Etiologically,  neuropathic  pre- 
disposition plays  in  any  event  the  chief  role.  Many  cases  affect  hysterical, 
psychasthenic,  psychotic  patients.  In  one  of  my  cases  there  was  a  history  of 
recent,  but  thoroughly  treated  syphilis  ( Wassermann  negative),  and  lues  is 
noted  in  a  number  of  cases  of  Raynaud's  disease  in  the  literature.  Nekam  and 
Curshmann  observed  its  family  occurrence.  As  exciting  causes,  acute  infec- 
tious diseases,  trauma,  severe  emotions  and  chilling  have  been  specially  men- 
tioned. 

The  disease  begins  usually  with  an  attack  of  "local  syncope";  one  or  several 
fingers  or  toes,  through  spasm  of  their  arterial  and  venous  vessels,  become  cold, 
waxy-pale,  and  so  ischemic  that  deep  needle  stabs  do  not  draw  any  blood.  Now, 
however,  I  must  warn  you  against  considering  every  local  syncope  of  this  kind 
as  a  forerunner  of  Raynaud's  disease.  Much  more  frequently  this  phenomenon 
("dead  fingers")  is  a  harmless  symptom  in  neurasthenics  and  other  psycho- 
neurotics, as  we  will  see  in  Lecture  XXATI,  also  in  Bright' s  disease.  Char- 
acteristic for  the  Raynaud  variety  of  this  symptom  is,  on  the  one  hand,  its 
appearance  in  symmetrical  positions  in  the  upper  or  lower  extremities,  on  the 
other,  the  (often  very  severe)  pains  which  introduce  or  accompany  it.  With 
continuation,  or  even  increase  of  these  pains,  there  is  added  to  the  stage  of 
local  syncope  which  is  of  short  duration,  from  minutes  to  hours,  that  of  "local 
asphyxia,"  which  lasts  considerably  longer  and  can  extend  over  many  hours, 
even  several  days.  On  the  fingers  or  toes,  and  indeed,  almost  exclusively  on 
the  end  phalanges,  there  appears  now  in  spots,  at  first  a  bluish,  then  a  con- 
tinually darker,  discoloration,  reaching  finally  slate-gray  in  color;  sometimes, 
besides  this,  there  are  extravasations  of  blood  under  the  skin  and  bullous  ele- 
vations of  the  epidermis.  Now,  in  the  favorable  cases,  the  circulation  is  es- 
tablished again,  when  a  decided  reddening  of  the  fingers  appears,  in  place  of 
the  regionary  cyanosis  ("stage  of  local  reddening")  ;  in  bad  cases,  however, 
the  local  asphyxia  of  the  affected  parts  passes  over  into  gangrene.  This  can 
be  total,  so  that  entire  phalanges  become  mummified  and  cast  off,  or  only 
partial,  in  which  necrosis  of  circumscribed  parts  of  the  skin  occurs.  Moist 
gangrene  is  quite  rare.  I  saw  it  in  the  case  with  luetic  previous  history  men- 
tioned above.  In  another  still  rarer  atypical  form  of  Raynaud's  disease,  not 
only  the  ends  of  the  extremities,  but  larger  or  smaller  portions  of  the  leg,  the 
buttock,  and  even  the  tip  of  the  nose,  the  lobe  of  the  ear,  etc.,  are  affected  by 
the  disease. 

The  relatively  mild,  that  is,  the  Raynaud's  paroxysms  not  coming  to  the 
gangrenous  stage,  can  be  repeated  over  years  or  decades,  then  at  length  to 
lead  to  mutilations.  Without  exception  the  general  condition  of  the  patient 
suffers  considerably  from  the  exceedingly  painful  attack.  As  a  regular  accom- 
paniment of  the  peripheral  vascular  spasm  there  is  to  be  mentioned  marked 
hyperesthesia,  which  makes  itself  evident  during  this  spasm  in  the  parts  affected; 
of  occasional  complications,  are  of  interest,  the  paralysis  of  the  sympathetic  of 
the  neck  (Horner's  symptom-complex),  further,  paroxysmal  disturbances  of  the 


SYMPATHETIC,    AXGIO-    AND    TROPHONEUROSES         377 

sense  organs,  which  may  well  depend  upon  vasomotor  spasm  (transitory  deaf- 
ness, blindness,  ageusia).  Here  belong  also  the  observations  of  transitory 
aphasia,  hemiglobinuria,  etc. 

The  course  can  be  quite  acute,  in  that,  after  one  or  a  few  attacks  the 
disease  comes  to  an  end  through  casting  off  of  the  affected  phalanges.  In  other 
cases,  however,  the  ever  returning  paroxysms  constitute  a  life-long  scourge. 
Life  itself  (if  we  leave  out  the  rare  cases  in  which  sepsis  has  occurred)  is  not 
threatened  by  angiospastic  gangrene. 

In  differential  diagnosis,  as  I  already  emphasized,  the  harmless  form  of 
"dead  finger"  is  to  be  thought  of.  A  benign  disease  is  also  presented  in  the 
"acrocyanosis  chronica  anaesthetica"  of  Cassirer.  In  this  affection,  an  asphyxia 
of  the  ends  of  the  extremities  develops  itself  little  by  little  and  is  accompanied 
by  paresthesias,  considerable  local  dulling  of  sensibility;  sometimes  also  by 
trophic  disturbances  (increase  of  volume).  The  distinction  of  this  from 
arteriosclerotic  "spontaneous  gangrene"  can  be  difficult  when  attacks  of  inter- 
mittent limping  or  intermittent  dyskinesia  of  the  upper  extremities  have  pre- 
ceded this  last  by  some  time.  Still,  the  angio-sclerotic  dyskinetic  paroxysms 
distinguish  themselves  from  those  of  Raynaud's  disease,  in  that  they  are  de- 
pendent upon  the  use  of  the  affected  extremity  and  disappear  upon  rest,  the 
accompanying  vasomotor  skin  changes  are  never  very  great  (a  spotted, 
marbled  condition  of  the  integument,  but  no  acro-cyanosis),  and  the  pulse  in 
the  peripheral  arteries  permanently  disappears  or  is  greatly  reduced.  The 
separation  of  Raynaud's  disease  from  "multiple  neurotic  gangrene  of  the  skin" 
in  which  on  different  portions  of  the  skin  after  a  painful  prodromal  stage,  or 
with  only  burning  and  prickling  paresthesias,  superficial  necrosis  takes  place, 
is  not  sharp.  In  making  this  diagnosis,  however,  the  greatest  caution  is  neces- 
sary. The  affection  attacks  very  frequently  hysterical  patients,  who  as  we 
will  see,  have  an  uncommon  tendency  to  self-mutilation;  in  one  of  mv  cases  by 
chemical  recognition  of  silver  in  the  sloughs,  I  was  able  to  prove  that  the  pa 
tient  had  produced  the  necrosis  with  nitrate  of  silver.  Mutilations  of  syringo- 
myelia and  lepra  are  separated  most  sharply  from  Raynaud's  disease  by  their 
painlessness. 

The  treatment  of  Raynaud's  disease  is  to  be  carried  out  after  the  analogy 
of  that  for  acroparesthesia  and  for  intermittent  limping  (see  above,  pages 
874-375),  only  one  will  do  well  to  avoid  all  faradic  applications,  which  here 
act  unfavorably.  Tin-  always  presenl  general  nervosity  must  lie  combated  with 
tlii'  greatest  care,  according  to  the  principles  to  he  mentioned  in  Lecture 
XXVIII;  they  must,  indeed,  he  considered  as  the  peculiar  substratum  of  the 
vasomotor  neurosis.  The  severe  pains  call  for  resort  to  the  most  active  aliti- 
neuralgics.      Sometimes   morphine    injections    cannot    lie   avoided. 

E.      Scleroderma 

This  disease,  which  is  usually  observed  in  women  of  middle  age  and  was 
described  for  the  first  time  by  Thirial,  is  much  more  frequenl  than  Raynaud's 
disease,  hut  still  must  he  denominated  a  rare  affection.  It  is  characterized  bj 
the  chronic  development   of  a    severe  trophic   alteration   of  the  skin   which  only 


378  LECTURE    XXV 

rarely  affects  in  a  diffuse  manner  the  whole  integument,  usually  is  limited  to 
definite  parts  of  the  face,  neck,  thorax  and  upper  extremities.  The  lower  half 
of  the  trunk  and  the  legs  usually  escape  the  disease.  Etiologically  we  know 
nothing  certain ;  the  same  points  which  we  brought  out  in  speaking  of  Ray- 
naud's disease,  could  be  repeated  here. 

The  sclerodermatically  altered  parts  of  the  skin  in  the  fully  developed  dis- 
ease, present  an  exceedingly  characteristic  appearance:  they  arc  thinned, 
smooth,  of  a  dull,  bacon-like  polish,  tense,  cool,  hardened  and  firmly  fixed  to 
the  subcutaneous  tissue.  Their  color  is  usually  pale,  sometimes,  however,  of  a 
yellowish  or  brownish  pigmentation,  also  vitiliginous  and  pigmented  spots  occur 
alongside  of  one  another.  Also  the  deeper  layers  of  tissue  (subcutaneous  fat, 
muscles,  tendons,  skeleton  of  the  hand)  undergo  an  increasing  atrophy.     Move- 


k      S*> 


> 


PI  k  L  J  >  i v 


P 


Fie.  105. 
Sclerodaetylism,  with  Formation  of  Necroses. 

ments  of  the  limbs,  opening  and  closing  of  the  mouth,  are  more  and  more 
hindered  by  the  skin  becoming  "too  tight.''  True  contractures  can  indeed 
occur. 

So,  diffuse  scleroderma  can  give  to  the  patient  a  mummy-like  appearance. 
As  to  the  circumscribed  forms,  they  furnish  a  great  variety  of  striking  clinical 
pictures.  The  alterations  usually  occur  more  or  less  bilaterally  and  symmetri- 
cally. They  are  found  particularly  frequently  on  the  fingers;  we  then  speak 
of  "sclerodaetylism";  with  this  there  are  also  atrophic  and  degenerative  pro- 
cesses in  the  nails,  occasionally,  indeed,  such  intense  disturbances  of  nutrition 
in  the  end  phalanges  that  actual  necroses  occur  (see  Fig.  105).  (Such  cases 
have,  in  my  opinion  incorrectly,  been  termed  a  combination  of  scleroderma  and 
Raynaud's  disease ;  as  a  diagnostic  criterion  of  the  last  affection,  in  any  case, 
the  painful,  paroxysmal  attacks  of  regionary  syncope  and  asphyxia  are  indis- 
pensable). Exceedingly  rare  is  the  "annular  sclerodaetylism"  of  During  which, 
distributed  in  a  circular  manner  can  lead  to  the  strangulation  of  the  phalanges. 


SYMPATHETIC,    AXGIO-    AND    TROPHONEUROSES         379 


The  diffuse  scleroderma  of  the  trunk  and  of  the  face  seldom  leads  to  round,  but 
usually  to  streak-like  foci  ("sclerodermic  en  bandes").  Particularly  typical 
are  isolated  streaks  extending  from  the  line  of  the  hair  to  the  eyebrows,  remind- 
ing one  of  the  scar  of  a  sabre  wound  ("sclerodermic  en  coup  de  sabre,"  see 
Fig.  106).  Also  the  mucous  membranes  can  be  affected  (mouth,  nose, 
pharynx).  Particularly  interesting,  though  also  very  rare,  are  the  observa- 
tions of  certain  circumscribed  sclerodermas  which  were  exactly  limited  to  the 
area  of  a  peripheral  nerve  or  to  a  spinal 
root  zone,  or  were  localized  on  one  side  of 
the  body  (Leu-ht,  Heller,  Bonn,  Brums, 
Curschmann,  and  others). 

As  preliminary  stages  of  the  atrophic 
changes,  a  stage  of  firm  oedema  and  an  in- 
durative stage  have  been  described;  still,  it 
appears  as  if  this  was  an  artificial  general- 
ization of  clinical  pictures  which  by  no 
means  occur  in  all  cases.  No  objective  dis- 
turbances of  sensibility  worth  mentioning 
occur  in  scleroderma,  on  the  contrary  the 
intact  appreciation  of  sensation  of  the 
greatly  altered  skin  (anatomically  it  is  a 
"cirrhosis"  of  the  chorium  with  thickening 
of  the  vessel  walls)  often  appears  to  us 
paradoxical.  Pains,  on  the  other  hand,  are 
not  rare,  particularly  in  the  hands.  Though 
they  also  sometimes  exacerbate,  still  they 
have  neither  the  intensity  nor  the  paroxys- 
mal character  of  those  which  are  peculiar 
to  Raynaud's  disease. 

Scleroderma  can  be  combined  with  different  nervous  diseases.  Particularly 
frequently  does  lliis  appear  to  be  the  case  with  regard  to  Basedow's  disease 
(observations  of  Leube,  Stahelim,  and  others)  which  all  the  more  attracts  at- 
tention to  its  eventual  connection  with  internal  secretory  processes,  as  Bruno 
Block  and  lieitmann  in  severe  scleroderma,  have  shown,  a  striking  variation 
of  the  nitrogen  balance  hit  ween  positive  and  negative  values;  pathological  in- 
crease of  albumin  destruction  is,  however,  as  we  saw  in  Lecture  XXIII,  a  typi- 
cal occurrence  in  Basedow's  disease.  Rarer  is  the  association  with  Addison's 
disease  or  tetany.  The  combination  with  Homer's  symptom  complex  i,  also 
quite  interesting;  the  patient  shpwn  in  Fig.  106  showed  this  last  on  the  side 
of  the  scleroderma  (in  the  picture  unfortunately  obscure).  In  the  most 
marked  case  of  diffuse  scleroderma  which  I  have  seen  (it  affected  a  13  year 
old    girl)    besides    this,    in    the    course    of    yens,    a    general    atrophy    of   all    the 

muscles  of  the  skeleton  developed;  also  during  the  progress  of  the  disease  ul 

ceis  and  fistulas,  from  which  carbonate  of  lime  was  discharged,  broke  out  in 
different  locations.  The  patient  died  of  marasmus.  The  examination  by 
Dietschg  showed  that  there  was  a  combination  of  scleroderma  with  interstitial 
polymyositis  and  calcareous  tendinitis. 


Scleroderma — "En    coup    de    sabre," 
with    Horner's   Symptom-Complex 

on  the  same  side-. 


380  LECTURE    XXV 

From  a  prognostic  point  of  view  it  is  to  be  remarked  that  a  cure  of  the 
changes  is  only  to  be  thought  of  in  the  earliest  stages,  when  no  atrophy  of  the 
skin  has  yet  occurred ;  later  lesions,  however,  are  to  be  considered  as  irrepar- 
able. Circumscribed  forms  in  a  location  which  does  not  cause  any  disturbance 
of  function  and  without  painful  phenomena,  usually  cause  no  difficulty  worth 
mentioning  and  can  become  stationary.  To  sclerodactylism,  however,  all  this 
does  not  apply  and  it  constitutes  a  severe  disease,  sooner  or  later  leading  to 
invalidism.  Exceedingly  extensive  forms  of  scleroderma  are  always  to  be  con- 
sidered as  very  serious ;  respiration  is  impeded,  the  act  of  eating  is  imperfectly 
performed  and  the  patients  die  in  cachexia,  if  they  are  not  taken  off  by  inter- 
current disease,  against  which  they  show  themselves  little  resistant.  Many  of 
these  patients  die  of  degeneration  of  the  heart,  muscle;  diffuse  scleroderma 
considerably  impairs  the  circulation  and  so  injures  the  heart. 

In  differential  diagnosis  it  may  be  remarked  that  "glossy  skin,"  as  it  occurs 
after  a  peripheral  nerve  lesion,  should  not  be  confused  with  scleroderma.  The 
other  skin  atrophies  (the  stria1  of  pregnancy,  senile  atrophy  of  the  skin,  simple 
and  pigmented  xeroderma)  have  no  resemblance  to  the  picture  of  scleroderma, 
since  in  them  all,  the  hardening  is  absent ;  a  similar  aspect,  however,  may  be 
presented  by  scars  (after  leg  ulcers,  burns,  lupus,  etc.)  ;  still  here  the  history 
will  naturally  exclude  wrong  diagnoses. 

In  treatment,  above  everything,  frequent  warm  baths  (eventually  sulphur 
baths,  mud  baths,  Fango  packs)  are  to  be  recommended;  also  the  steam  bath 
and  the  hot  air  bath  ("Bier's  box'')  can  be  tried.  Mild  massage  is  nearly  al- 
ways found  beneficial.  Of  internal  medication  the  salicylic  preparations  ap- 
pear to  exercise  a  decided  effect,  (sodium  salicylate,  acetylo-salicylic  acid,  salol, 
etc.)  ;  also  ichthyol  capsules  (ail  0.25,  1  to  3  capsules  t.  i.  d.)  have  been  recom- 
mended. Ichthyol,  by  the  way,  comes  also  into  external  application,  as  does 
thiosinamine  plaster.  Hcbra  has  introduced  thiosinamine  into  the  therapy  also 
in  the  form  of  injections  (every  second  day  0.5  cc  of  a  15  per  cent,  alcoholic 
solution).  These  injections  are  very  painful;  better  tolerated  is  the  nearly 
related  fibrolysin,  which  Cursch/mann  has  warmly  recommended  upon  the 
ground  of  favorable  experiences.  Also  courses  of  iodide  appear  to  me  often 
to  act  favorably.  For  the  sclerodermic  pains  I  recommend  the  following  mix- 
ture, which  must  be  shaken  up : 

R<      Sodii  iodid, 

Lactophenin aa  5.0  (gr.   75) 

Codein  phosph 0.2  (gr.  3) 

Spirit  vmi    20.0  (5  5) 

Infus.  radic.  valerian  (10  per  cent.)    ad  150.0  (§   5) 

M.  S.     Three  tablespoon  fills  a  day  until  the  pains  are  relieved,  then  one 
or  two  as  needed.        Shake  before  using. 


SYMPATHETIC,    AXGIO-    AXU    TROPHONEUROSES         381 


F.      Neurotic  Dropsy 

Exudations  of  scrum  occurring  from  angio-  or  tropho-ncurotic  cause  we 
meet  with  in  two  different  forms,  on  the  one  hand  as  neurotic  oedema  of  the 
skin,  on  the  other  as  neurotic  dropsy  of  the  joints. 

1.  Circumscribed  (Edema,  of  the  Skin  (Quincke). — This  affection  is  rela- 
tively rare  and  occurs  in  young  individuals  of  nervous  predisposition.  (Pre- 
dominance in  the  male  sex  is  plainly  evident.)  Partly  in  connection  with  ex- 
citing emotions,  trauma,  exposure  to  cold,  partly  without  recognizable  ex- 
ternal cause,  sometimes  hereditarily,  circumscribed  swellings  of  the  skin  which 
have  a  doughy  feeling  and  retain  the  impression  of  the  finger,  occur  in  attacks. 
Their  color  is  pale  or,  on  the  contrary,  somewhat  reddened,  in  size  they  vary 
from  that  of  a  dollar  to  that  of  the  palm  of  the  hand.  Multiplicity  of  these 
usually  quite  indolent,  but  sometimes  itching  urticaria-like  disease  foci,  is  fre- 
quent. They  can  occur  at  any  point  on  the  surface  of  the  skin,  also  may  ex- 
tend to  the  mucous  membrane  of  the  mouth,  the  pharynx  and  the  conjunctiva. 
The  seat  of  predilection  is  the  face,  after  this,  the  backs  of  the  hands.  The 
appearance  of  the  oedema  is  sometimes  accompanied  by  vomiting  and  diar- 
rhoea which  has  been  considered  as  an  expression  of  neurotic-cedematous  changes 
in  the  intestinal  tract;  in  a  case  of  my  observation  (which  occurred  in  a  pre- 
viously healthy  girl  after  the  railroad  accident  at  Miillheim  in  Baden)  the 
attacks  were  at  the  start  accompanied  by  bloody  diarrhoea.  Joseph  has  ob- 
served hemoglobinuria.  After  a  few  hours  the  circumscribed  oedema  disap- 
pears again,  but  has  a  great  tendency  to  recur.  Periodic  (also  menstrual) 
appearance  is  sometimes  observed.  Many  patients  suffer  during  their  whole 
Eves  from  this,  as  a  rule,  harmless  affection,  which  can  only  be  dangerous  when 
it  is  localized  at  the  entrance  to  the  larynx.  Still,  permanent  recoveries  are 
not  entirely  rare.  There  are  certainly  close  nosological  relations  between 
Quincke's  (edema  and  chronic  recurrent  giant  urticaria.  Many  patients  suffer 
simultaneously  from  Quincke's  oedema  and  urticaria;  in  one  case  of  Bircher's 
an  urticarial  eruption  regularly  preceded  the  attack  of  (edema  and  indeed,  so 
that  the  two  affections  always  appeared  at  different  locations  on  the  skin.  Also 
Combination  with  asthma  and  migraine  attacks  occurs.  The  pathogenesis  of 
the  disease  is  obscure;  perhaps  it  is  (he  result  of  a  regional  venous  spasm. 
Umrjlcr  as  physician  to  the  Basle  house  of  correction,  has  observed  a  regular 
guesl  of  international  penal  institutions,  who  (in  order  to  avoid  punishment) 
could  produce  at  will  circumscribed  (edema  of  the  face,  but  who  would  not  dis- 
close his  method  of  procedure-;,  Hv/nziker  suspected  that  it  was  by  manual 
Compression  of  a  venous  trunk.  On  the  other  hand  Stahelin  has  shown  that. 
eastro  intestinal  autointoxication  may  play  a  role  as  exciting  cause  for  the 
appearance  of  Quincke's  oedema,   as   is  so   often    the   c:r~>-   In   urticaria. 

Still  more  obscure  is  the  pathogenesis  of  the  so  called  "chronic  form  of 
neurotic  oedema."  By  this  is  understood  hydropic  swellings  of  the  skin,  usually 
located    on    the    leg   or    on    the    forearm,    which    appear    without    any    apparent 

cause,  also  an-  observed   as  a    family  complaint    ("trophoedeme   familial"  of 

Mcii/i  )    and    last    for  years   or   for   life,   without    any   organic    (renal    Or   cardio- 


382  LECTURE    XXV 

vascular)  changes  being  present.  You  are  warned  against  confusing  traumatic 
forms  with  the  so-called  "hard  traumatic  oedema."  This  last,  when  it  appears 
upon  the  backs  of  the  hands,  is  often  the  result  of  fraudulent  manipulations 
of  skilled  simulators,  as  Secretan  and  Haegler  have  recognized. 

Therapeutically,  regular  saline  purgation,  bland  and  lacto-vegetarian  diet, 
daily  cold  spongings,  rain  douches  or  river  baths,  are  to  be  advised.  Of  medi- 
cations I  would  recommend  long-continued  administration  of  salicylate  of 
quinine  (0.25  twice  a  day)  to  be  tried  in  every  case.  Threatened  obstruction 
of  the  entrance  of  the  larynx,  danger  of  suffocation  has  on  various  occasions 
rendered  tracheotomy  necessary.  After  this  the  application  of  a  permanent 
tracheotomy  tube  is  recommended. 

2.     INTERMITTENT    DROPSY    OF    THE    JOINTS 

Still  more  rare  than  Quincke's  oedema  is  intermitting  dropsy  of  the  joints 
(Moore).  This  is  a  periodic  swelling  of  the  joints,  sometimes  recurring  with 
striking  chronological  regularity,  which  usually  affects  one,  somewhat  more 
rarely,  both  knee-joints.  In  one  of  my  patients  the  articular  dropsy  occurred, 
for  example,  every  13  days.  The  patient  was,  however,  during  these  intervals 
free,  but  in  them  he  suffered  from  cyclic  melancholia  (also  in  cases  of  Reismger 
and  Morris,  there  was  a  13-day  rhythm).  Intermittent  dropsy  of  the  joints, 
apart  from  a  troublesome  feeling  of  tension  in  the  joints,  runs  its  course  with- 
out important  subjective  symptoms. 

Still,  sometimes  disagreeable  accompanying  symptoms  have  been  mentioned 
(slight  fever,  palpitation  of  the  heart,  vertigo,  vomiting,  hyperidrosis,  poly- 
uria, diarrhoeas,  migraine,  etc.).  After  from  1  to  3  days  the  swelling  dis- 
appears again. 

The  treatment  coincides  with  that  of  Quincke's  (edema  ;  also  we  can  repeat 
the  little  that  we  have  said  about  the  etiology  of  this  last  here.*  A  difference 
consists  in  the  fact  that  neurotic  joint  dropsy  attacks  by  preference  the  female 
sex,  from  which  it  can  be  concluded  that  physical  overexertion  plays  no  special 
etiological  role.  The  prognosis  is  worse  than  in  circumscribed  oedema  of  the 
skin,  permanent  recovery  still  rarer. 

G.      Erythromelalgia 

Erythromelalgia.  which  was  first  described  by  Weir  Mitchell,  represents 
the  type  of  a  vaso-dilator  neurosis  of  the  extremities.  Still,  I  have  seen  the 
disease  in  a  case  running  a  favorable  course  and  of  otherwise  typical  char- 
acter, appear  in  the  face,  which  must  be  denominated  "erythroprosopalgia." 
The  very  rare  disease  occurs  almost  exclusively  in  grown  persons  and  mani- 
fests a  certain  predilection  for  the  male  sex.  Those  affected  are  generally 
persons  of  neuropathic  heredity  and  as  exciting  causes,  exposure  to  cold  and 
overexertion  have  been  accused.  The  upper  extremities  are  much  more  rarely 
attacked  than  the  lower,  in  which,  in  typical  cases,  the  "erythromelalgic  at- 
tacks" first  manifest  themselves  by  severe  neuralgiform  pains.     Soon  there  fol- 

*  No  surgical  treatment.    It  cannot  aid,  but  will  injure. 


SYMPATHETIC,    ANGIO-    AND    TROPHONEUROSES         383 

lows  an  intense  reddening  of  the  toes,  of  the  feet,  sometimes  also  of  a  part  of 
the  leg,  in  rare  eases  indeed,  of  the  whole  lower  extremity;  this  reddening, 
which  is  usually  accompanied  by  swelling,  is  sharply  separated  from  the  normal 
parts  of  the  skin.  With  the  appearance  of  this  local  dilatation  of  the  vessels, 
the  pain  usually  somewhat  diminishes  in  intensity.  The  affected  parts  of  the 
skin  feel  hot  and  sometimes  sweat  profusely;  they  are  the  seat  of  a  decided 
hyperesthesia.  The  erythromelalgic  attack  can  last  hours,  days,  or  even  weeks ; 
in  the  last  case  the  flaming  redness  more  and  more  gives  way  to  a  cyanotic 
coloration  and  the  local  hyperthermia  subsides.  In  many,  perhaps  most  cases 
of  erythromelalgia,  between  the  individual  attacks  the  integument  of  the  af- 
fected parts  does  not  completely  return  to  normal,  but  there  remains  a  certain 
degree  of  vaso-dilatation  along  with  different  trophic  disturbances  which  per- 
sist interparoxysmally  ;  this  is  called  "'chronic  erythromelalgia."  These  chronic 
cases  are  prognostically  very  unfavorable,  while  true  paroxysmal  forms  can 
recover.  Erythromelalgia,  indeed,  does  not  constitute  a  danger  to  life.  The 
differential  diagnosis  from  erysipelas,  acute  gout,  inflammatory  flat-foot,  etc.} 
is  often  quite  difficult.  As  erythromelia  is  denominated  a  painless,  vaso-dilator 
neurosis,  which  has  been  observed  on  the  extensor  side  of  the  limbs.  As  treat- 
ment during  the  attack,  elevation  of  the  affected  extremity  witli  cold  com- 
presses, is  to  be  recommended  (while  a  hanging  position  and  warmth  consider- 
ably increase  the  distress.  Further,  naturally,  the  use  of  different  anti- 
neuralgics.  Electro-therapy  is  of  little  or  no  use.  In  hopeless  cases  nerve 
resection,  and,  indeed,  amputation,  have  been   carried  out. 

H.     Facial  Hemi-atrophy  and  Hemi-hypertrophy 

Both  these  peculiar  trophic  neuroses,  especially  the  second  mentioned,  are 
very  rare,  and  for  this  reason,  particularly,  however,  on  account  of  their  thera- 
peutic intractability,  are  practically  without  importance.  To  complete  our 
discussion  though,  we  will  sketch   I  hem   in   their  outlines. 

Progressive  facial  hemi-atrophy,  or  Romberg's  disease,  affects  chiefly  fe- 
males in  early  life  and  is  usually  introduced  by  neuralgiform  pains  in  the 
affected  half  of  the  face  which  sometimes  are  also  present  in  the  fully  developed 
stage  of  the  affection.  Very  gradually  there  develops  a  strictly  unilateral 
atrophy  of  the  skin  and  of  the  subcutaneous  connective  and  fatty  tissue,  often 
also  of  the  bones  of  the  face  and  of  a  few  muscles  (the  temporals,  masseters, 
tongue  muscles).  One-half  of  the  face  hence  sinks  in,  in  a  striking  manner.  As 
rarities,  cases  have  been  described  in  which  the  atrophy  has  extended  also  to 
the  neck,  the  thorax  and  the  arm.  In  the  majority  of  cases  the  circumscribed 
Atrophy  of  the  face  affects  the  left  side.  Sensibility  remains  unaffected  in 
tin'  atrophic  region;  on  the  other  hand,  in  it  vitiligo,  abnormal  pigmentation, 
falling  nut  of  the  hair,  decolori/a  I  ion  of  the-  hair,  etc.,  are  occasionally  ob- 
served. Therapeutically,  the  disease  is  not  to  be  influenced;  it  can,  however, 
spontaneously  come  to  a  standstill  before  the  deformity  has  reached  a  very 
high  degree.  In  these  last  cases  cosmetic  paraffin  prosthesis  hy  Gersu/ny'a 
method  has  sometimes  given  good  results.  Pathogenetically  Romberg's  disease 
is  one  of  tin-  most   obscure  of  the  tropho-neuroses.     A   near  relationship   t'» 


384 


LECTURE    XXV 


scleroderma  is  the  more  probable,  since  combinations  of  both  pathological  con- 
ditions occur. 

The  contrary  condition  to  facial  hemi-atrophy,  facial  hemi-hypertrophy 
was  first  described  by  Friedreich  in  the  year  1862.  It  is  such  a  rare  disease 
that  the  last  reviewer  of  the  subject.  Wanner,  up  to  1908,  could  find  only  29 
cases  in  the  literature.  It  consists  in  a  considerable  increase  in  volume  of  the 
soft  parts,  sometimes  also  of  the  bones,  on  one  side  of  the  face,  including  the 
forehead  and  parietal  region ;  the  tongue,  the  ear,  the  tonsil  can  take  part  in 
the  hypertrophy.  Anomalies  of  pigmentation,  vasomotor  disturbances,  uni- 
lateral exophthalmus,  and  other  things  can  complicate  the  picture.  Men  and 
women  are  found  with  equal  frequency  among  those  affected.  Contrary  to 
facial  hemi-atrophy,  the  disease  most  frequently  affects  the  right  side.  Neural- 
giform symptoms  are  rarer  than  in  Romberg's  disease. 

I.    Herpes  Zoster 

Herpes  zoster  ("Gurtelrose,"  "Zona"  of  the  French  authors)  is  a  typical 
vesicular  eruption  which  in  its  topographical  distribution   appears  connected 


Fig.  107. 
Herpes  Zoster  of  the  Trunk. 


with  the  segmentary  areas,  that  is,  with  the  regions  of  distribution  of  the  pos- 
terior spinal  roots  or  their  cranial  homologues.  Fig.  107  represents  a  herpes 
zoster  on  the  trunk,  Fig.  108,  one  on  the  face.  In  the  last  case  I  beg  you  to 
note  that  the  herpetic  area  does  not  correspond  exactly  with  the  distribution 


SYMPATHETIC,    ANGIO-    AND    TROPHONEUROSES 


38.5 


of  a  single  branch  of  the  trigeminus,  but  surrounds  the  nose  more  or  less  con- 
centrically and,  hence  affects  the  region  of  all  three  divisions.  It  is  indeed,  a 
radicular,  not  a  peripheral-nervous  tropho-neurosis.  Barensprung's  investiga- 
tions, which  have  been  confirmed  by  numerous  later  authors,  have  shown  that 
the  seat  of  the  disease  is  to  be  sought  in  the  spinal  ganglia,  respectively  in  the 
homologous  structures  of  the  sensory  brain  nerves  (  particularly  in  the  Gasserian 
ganglion).     It  may  be  an  acute  inflammation  of  these  (according  to  Head  and 


Fig.  108. 
Herpes  Zoster  of  the  Face  (with  Homer's  Symptom-Complex). 

(  a  in  pbell,  usually  so),  but  also  may  In-  due  to  tumors,  trauma,  etc.  The 
physio-pathological  hasis  of  tin-  vesicular  eruption  i^  indeed  obscure  to  us  in 
spite  of  this  topical  diagnostic  recognition.  In  the  mechanism  concerned,  how- 
ever, connection  with  the  sympathetic  comes  into  question.  Of  importance  in 
this  relation  is.  for  example,  the  condition  that  in  the  patient  shown  in  Fig. 
108  a  mvosis  and  enophthahnus  and  the  narrowing  of  the  palpebral  fissure  oc- 
curred on  the  same  side  as  the  herpes  eruption.  Etiological!;  in  the  so-called 
secondary  cases,  as  already  said,  then'  have  been  injuries  to  the  ganglia  (frac- 
ture of  the  vertebral  column,  etc.)  or  tumors  (carcinoma  metastases,  for  ex- 
ampli  ):   further,  poisoning  with  carbonic  oxide  and   arsenic   should   be   men- 


386  LECTURE    XXV 

tioncd.  With  regard  to  the  last,  there  has  been  a  controversy  as  to  whether 
the  herpes  occasionally  observed  after  salvarsan  injections  is  to  be  considered 
as  a  toxic  manifestation  or  as  the  so-called  "Neurorecidiv"  of  syphilis.  In 
primary  or  idiopathic  herpes  zoster,  which  we  will  now  exclusively  consider, 
there  appears  to  be  an  acute  infectious  disease  sui  generis,  in  which  exposure 
to  cold  and  similar  things  can  only  be  considered  as  exciting  causes;  the  actual 
cause,  however,  is  indeed  still  unknown  to  us.  For  this  speak  the  above-men- 
tioned pathological  findings  of  Head  and  Campbell,  further  the  facts  that  the 
disease  appears  to  leave  behind  it  an  immunity,  runs  its  course  with  fever, 
swelling  of  the  glands,  and  pleocytosis  of  the  cerebrospinal  fluid,  and  last,  but 
not  least,  its  occasionally  observed  appearance  in  epidemics. 

In  typical  cases  of  idiopathic  zoster  a  moderate  rise  of  temperature  (to 
about  39°  C.  (102.2°  F.)  with  general  discomfort,  disturbances  of  digestion, 
etc.,  precedes  the  eruption.  With  this  there  is  usually  very  severe  burning  and 
boring,  sometimes  continued,  sometimes  exacerbating  pains  in  the  affected  radi- 
cular area ;  these  pains  can  appear  simultaneously  with  the  vesicular  eruption^ 
or  indeed,  only  after  its  occurrence.  They  usually  get  worse  toward  evening- 
The  herpes  eruption  itself  is  usually  introduced  by  the  appearance  of  red 
spots  upon  a  normal  appearing  ground.  Soon,  however,  the  epidermis  over 
these  spots  raises  itself  to  serous  vesicles  whose  contents  gradually  become 
cloudy  and  purulent.  If  the  sensibility  of  the  skin  in  the  neighborhood  of  the 
vesicles  is  tested,  sometimes  hypesthesia  and  anesthesia,  more  rarely  hyper- 
esthesia, is  found.  Sometimes  atypical  forms  of  eruption  appear:  bullous, 
hemorrhagic,  gangrenous,  ulcerative  herpes ;  the  last  two  may  leave  behind 
scars. 

Regional  glandular  swellings  are  not  rare,  also  hyperidrosis  or  anidrosis 
occur,  as  do  also  obstinate  neuralgias,  which  may  persist  after  the  herpes  has 
recovered,  which  takes  usually  from  2  to  3  weeks,  but  sometimes  considerably 
longer.  Such  persistent  root  neuralgias  cloud  the  prognosis  of  the  in  general 
entirely  mild  disease,  especially  in  decrepit  old  people,  who  form  a  large  contin- 
gent of  the  zoster  patients.  Serious  results  may  also  follow  an  extension  of  the 
herpes  zoster  to  the  cornea,  when  it  may  lead  to  ulceration  and  panophthal- 
mitis.    Still,  this  localization  is,  fortunately,  not  frequent. 

Therapeutically,  we  limit  ourselves  to  the  administration  of  antineuralgics 
(see  Lecture  III,  page  57),  and  the  application  of  a  2  to  5  per  cent,  anes- 
thesin  ointment,  or  of  a  powder  of  talc,  bismuth  subnitrate  and  starch,  equal 
parts.  Ulcerated  herpes  vesicles  heal  best  under  5  to  10  per  cent,  ointment  of 
balsam  of  Peru.  Ophthalmic  herpes  should  be  turned  over  to  the  ophthal- 
mologist without  delay. 

Gentlemen:  We  will  here  break  off  our  sketching  of  the  most  typical 
tropho-vasomotor  syndromes.  I  must  remark  to  you,  however,  that  it  is 
just  in  this  class  of  cases  that  mixed  and  transition  forms  are  specially  fre- 
quent ;  for  instance,  not  only  can  the  different  vaso-constrictor  conditions  be 
combined,  but  also  vaso-constrictor  and  vaso-dilator  neuroses  have  been  ob- 
served in  one  and  the  same  patient,  for  instance,  erythromelalgia  and  Raynaud's 
disease. 

It  may  be  also  related  that  many  skin  diseases  by  their  topographic  limita- 
tion to  definite  regions  of  innervation,  symmetric  distribution,  etc.,  proclaim 


SYMPATHETIC,    ANGIO-    AND    TROPHONEUROSES         387 

themselves  of  tropho-neurotic  or  vaso-neurotic  nature.  I  would  recall  many 
vascular  n;evi,  cases  of  vitiligo,  partial  albinism,  pigmented  moles,  etc.  Also, 
symmetric  lipomatosis  or  Madelwng's  disease  may  will  belong  here  (see  Fig. 
109).  As  a  variety  of  this  last  is  to  be  considered  the  adipositas  dolorosa 
or  Dcrcum's  disease,  which  also  shows  its  connection  with  nervous  diseases, 
particularly  by  the  element  of  pain.    It  affects  mainly  women  (often  alcoholics) 


Fib.  109. 
,\l,iih  hunt's  Disease. 

who.  also  in  general  very  corpulent,  present  tumor-like  lumps  of  fat  in  the  sub 
cutaneous  tissue  of  the  arm,  feg  and  trunk,  but  with  regular  escape  of  the 
hands  and  feet.  These  lumps  of  fat  are  sensitive  to  pressure,  as  are  also  the 
regional  nerve  trunks.  Also,  spontaneous  pains  may  arise  in  these  [ipomata. 
Anesthesia  or  hyperesthesia  of  the  affected  skin  zones  are  occasionally  noted. 
The  attempt  of  a   few  authors  to  prove  the   relationship  between   Den  urn's  dis 

ease  and  mv xcedema  iniisi  be  considered  as  a  failure.     Finally,  progressive  lipo 
dystrophy  in  which  the  upper  half  of  the  body  emaciates  to  complete  loss  of 
the  panniculis  adiposus  while  the  lower  hull',  on  the  contrary,  shows  rather  an 
increased  deposition  of  fat.  i^  to  be  mentioned. 


LECTURE  XXVI 
Epilepsy 

Gentlemen  :  The  disease  to  which  to-day's  lecture  is  devoted,  must  be 
considered  as  the  first  nervous  disease  of  which  literary  information  has  been 
transmitted  to  us.  That  it  since  most  remote  times  has  laid  claim  to  the 
phantasy  of  physicians  and  laymen  to  a  high  degree  and  that  it,  as  no  other 
disease,  has  had  the  reputation  of  being  of  supernatural  origin,  we  need  not 
wonder.  Indeed,  the  "falling  sickness"  which  causes  a  man  apparently  in  perfect 
health,  to  fall  unconscious  and  to  go  into  convulsions,  must  make  upon  the 
masses  of  the  people  a  particularly  alarming,  indeed,  uncanny  impression. 
Even  the  "scientific"  name  epilepsy,  which  we  have  inherited  from  ancient  times, 
indicates  nothing  but  "being  seized"  or  "possessed."  You  know,  indeed,  that 
the  disease  was  considered  as  a  punishment  inflicted  by  the  gods,  hence  the  name 
customary  to  the  Hippocratic  body,  "hpij  vo<ro?"=sacred  disease.  "Morbus 
sacer"  is  still  occasionally  used  to-day.  Just  as  respectable  is  also  the  age  of 
the  designation  popular  in  France,  "mal  comitial"  or  "crises  comitiales." 
Namely  in  ancient  Rome  if  during  the  Comitia  some  one  had  an  epileptic  fit, 
on  account  of  this  "morbus  comitialis,"  the  assembly  had  to  be  closed  imme- 
diately. 

We  speak  to-day  expressly  of  a  genuine,  essential,  or  idiopathic  epilepsy, 
since  we  very  properly  assign  to  the  epileptiform  symptoms  as  they  have  been 
observed  in  the  most  varied  pathological  conditions  of  the  nerve  centers — for 
example,  multiple  sclerosis,  progressive  paralysis,  cerebral  syphilis,  cerebral 
abscess,  cerebral  tumor,  hydrocephalus,  infantile  cerebral  palsy,  meningitis, 
saturnism,  alcoholism,  morphinism,  etc. — a  special  position  as  "symptomatic 
epilepsies."  We  have,  indeed,  in  the  course  of  these  lectures,  become  ac- 
quainted with  most  of  these  symptomatic  epileptic  phenomena  and  in  our 
further  remarks  will  only  consider  true  epilepsy.  This  may  be  defined  in  the 
following  manner:  A  chronic,  very  frequently  progressive,  disease  of  the  brain, 
which  manifests  itself,  as  a  rule,  by  more  or  less  frequent  convulsive  attacks 
accompanied  by  loss  of  consciousness  (sometimes,  however,  by  paroxysmal  dis- 
turbances of  consciousness  without  convulsions),  and  which  not  rarely  can 
lead  lo  psychical  alteration.  It  appears  sometimes  even  in  early  childhood, 
usually,  however,  between  puberty  and  the  20th  year  of  life.  Numerous,  how- 
ever, are  the  cases  of  the  last  category  in  which  we  learn  in  the  history  that 
the  affected  patients  as  infants  have  suffered  from  "tooth  spasms,"  infantile 
eclampsia,  and  one  cannot  escape  the  impression  that  these  convulsive  par- 
oxysms, originally  considered  as  harmless,  were  really  the  precursors  of  epi- 
lepsy.    The  beginning  of  true  epilepsy  in  the  third  and  fourth  decade  is  less 

388 


EPILEPSY  389 

frequent,  in  still  later  years  quite  rare.  An  epileptic  attack  occurring  for  the 
first  time  beyond  the  40th  year,  in  the  majority  of  cases  is  of  the  symptomatic 
form  ( progressive  paralysis,  brain  tumor,  brain  syphilis,  etc.).  Both  sexes 
are  attacked  with  about  equal  frequency. 

Symptomatology 

We  will  begin  our  sketch  of  the  epileptic  disease  picture  with  a  description 
of  the  typical  convulsive  attack,  then  will  subject  to  clinical  consideration, 
paroxysms  of  other  sorts  and,  finally,  the  permanent  interparoxysmal  anomalies 
of  epilepsy. 

1.     The  Severe  Epileptic  Convulsive  Attack 
("Haut  mal,"  Epilepsia  Major,  Convulsiva) 

The  epileptic  convulsion  can  seize  the  patient  without  an}'  warning,  "like 
lightning  out  of  a  clear  sky";  still,  in  many  cases,  it  begins  with  prodromal 
symptoms  which  we  denominate  the  epileptic  "aura"  and  whose  different  types 
we  will  now  enumerate.  It  occurs  not  at  all  infrequently  that  even  before  the 
aura  (which  is  always  of  very  short  duration,  seconds  or  minutes)  different 
disturbances  of  the  general  condition  announce  the  impending  attack.  So, 
many  patients  complain  for  several  hours  or  even  days  previously,  of  sense 
of  pressure  in  the  head,  malaise,  abnormal  irritability,  sexual  erethism,  sleep- 
lessness, urticaria,  diarrhoea,  frightful  dreams,  abnormal  appetite,  and  other 
anomalies. 

The  epileptic  aura  can  be  sensible,  sensory,  motor,  vasomotor  or  psychic. 
The  first  is  the  most  frequent.  The  feeling  of  a  cold  or  warm  breeze  which 
passes  over  the  body  surface  of  the  patient  has  given  occasion  for  the  choice 
of  tli.'  term  "aura"  (air)  which  dates  back  to  the  time  of  Galen.  Further, 
paresthesias  of  the  ends  of  the  extremities,  of  the  tongue,  of  the  scalp  have 
been  described,  also  a  feeling  of  constriction  in  the  throat,  stabbing  pain  in 
!li<-  head,  etc.  The  sensory  aura  can  affect  the  various  sensory  organs,  far 
most  frequently  it  is  an  optic  aura.  The  patient  suddenly  perceives  dark 
spots,  scotomata,  in  the  visual  field,  or,  on  the  contrary,  flames,  lightning, 
sparks,  balls  of  fire,  sees  everything  in  one  definite  color  (almost  always  red) 
the  so-called  "colored  vision."  or  in  altered  size  (macropsia  and  micropsia), 
etc.  The  auditory  aura  is  usually  characterized  by  a  roaring,  cracking,  or 
hissing  iii  our  or  both  ears,  the  olfactory,  by  a  penetrating  smell,  the  gustatory 
l.v  a  bitter  or  astringent  taste.  In  the  motor  aura  there  occur,  for  example, 
twitching  iii  the  facial  distribution,  choreiform  movements  of  the  hands,  singul 
tus,  yawning  spasms,  motor  aphasic  symptoms,  purposeless  running  forward 
( "aura  cursatoria")  etc. 

As  vasomotor  aural  phenomena,  paling,  reddening,  outbreak  of  sweat  and 
palpitations  occur,     finally,  among  other  things   there  are  designated   as  "psy- 

chic  aura."  the  >udden  causeless  appearance  of  a  d<  finite  memory,  often  » ith  the 

plainness  of  a  hallucination,  or  an  abrupt   change  of  i I.  for  instance,  an 

outbreak  of  mirth,  or   feelings  of  anxiety   and   imperative   idea-.     Often    lb'' 


390  LECTURE    XXVI 

aura  is  a  complex  one  made  up  of  psychic,  sensor}',  sensible  and  motor  com- 
ponents in  manifold  combination,  but  it  usually,  in  one  and  the  same  patient, 
repeats  itself  in  a  thoroughly  stereotyped  manner,  so  that  he  is  entirely 
aware  of  the  threatening  attack.  Indeed,  the  aura  is  so  short  that  in  but 
very  few  cases  the  epileptic  finds  time  to  take  adequate  precautions,  for  ex- 
ample, to  prevent  falling  by  lying  down. 

Whether  an  aura  precedes  it  or  not,  the  epileptic  attack  proper  begins  in 
the  most  abrupt  and  "brutal"  manner.  In  typical  cases  the  patient  emits  a 
penetrating  cry  and  falls  unconscious,  while  wounds  from  striking  the  head 
or  the  limbs  or  from  falling  down  stairs,  etc.,  upon  which  the  patient  is  at 
the  time,  are  nothing  rare.  Biting  the  tongue  occurs  from  the  sudden  bring- 
ing together  of  the  teeth,  and  in  one  of  my  cases  this  led  to  almost  complete 
hemisection  of  that  organ.  Now  a  general  tonic  spasm  of  the  muscles  appears, 
the  jaws  are  pressed  together,  the  fists  clenched,  arms  and  legs  are  rigidly 
extended,  the  eyes  are  directed  fixedly  forward  or  are  drawn  up  under  the 
fast-closed  lids,  the  pupils  are  usually  narrowed  and  reactionless ;  also  the 
inspiratory  muscles  usually  take  on  a  tonic  contraction  and  respiration  is 
suspended.  Hence,  the  sudden  pallor  of  the  face,  which  is  usually  observed 
in  epileptics  at  the  beginning  of  the  attack  passes  rather  quickly  over  into  a 
livid,  indeed  cyanotic  color.  This  "tonic  stage"  of  the  severe  epileptic  attack 
is  of  very  short  duration  (about  one-half  minute)  and  passes  over  into  the 
"clonic  stage,"  which  usually  lasts  from  several  minutes  to  one-quarter  hour. 
The  limbs  undergo  violent  contractions,  the  body  writhes,  the  eyes  roll  wildly, 
the  countenance  is  drawn  up  into  frightful  grimaces,  the  head  is  thrown  in  all 
directions.  Also  in  this  stage  biting  the  tongue  and  other  wounds  can  be  pro- 
duced. There  occurs  also  an  excessive  secretion  of  saliva  which  appears  upon 
the  lips  as  a  blood-tinged  foam.  The  pupils  are  still  rigid,  but  now,  however, 
maximally  dilated.  The  respiration  is  stertorous  and  can  be  heard  at  a  dis- 
tance ;  outbreak  of  sweat  and  discharge  of  urine  are  frequent,  rarer,  discharge 
of  faeces  and  ejaculation.  The  face  appears  dark  red,  swollen;  the  veins  of 
the  neck  are  congested ;  sometimes  there  are  small  extravasations  of  blood  into 
the  conjunctiva,  on  the  neck,  on  the  chest,  behind  the  ears,  etc.  Now  follows, 
with  gradual  cessation  of  the  convulsions,  passage  into  a  quiet  coma,  in  which 
the  stertorous  breathing  little  by  little  changes  into  the  normal  type  of  respira- 
tion and  the  cyanosis  again  gives  place  to  pallor.  This  last  stage  of  the  attack 
can  be  of  very  short  duration,  but  may  last  from  one  to  two  hours;  then  the 
patient  slowly  regains  his  senses.  As  a  rule,  for  hours  he  feels  weak,  exhausted, 
depressed,  suffers  from  severe  headache,  sometimes  also  from  nausea  and 
vomiting.  There  is  complete  amnesia  for  the  attack;  indeed,  the  memory  of 
the  aura  can  at  times  be  absent.  Sometimes  the  temperature  is  somewhat 
elevated,  often  there  is  slight  albuminuria  which  soon  disappears.  Very  rarely 
transitory  paralyses  are  to  be  observed  in  this  post-paroxysmal  stage,  most 
frequently  convergent  strabismus.  After  one  or  two  days  they  usually  dis- 
appear. Also  there  are  occasionally  temporary  pareses  of  one  arm  or  leg, 
or  of  the  two  extremities  on  the  same  side:  on  two  occasions  I  could  elicit 
BabinskVs  toe  phenomenon  (once  also,  ankle  clonus).  Further,  post-parox- 
ysmal transitory  aphasia  as  well  as  fleeting  sensory  paralyses   (post  epileptic 


EPILEPSY  391 

deafness  and  blindness)  need  to  be  mentioned.     Mushens  has  found  after  epi- 
leptic attacks  cutaneous  hyperesthesias  of  segmental  type. 

That  a  true  epileptic  attack  occurs  unilaterally  ("genuine  hemi-epilepsy" ) . 
that  is.  like  the  Jacksonian  attacks  sketched  in  Lecture  XIX  (page  293),  is 
exceedingly  rare.  In  order  to  include  such  cases  within  the  limits  of  the  mor- 
bus sacer,  in  spite  of  the  atypical  nature  of  the  convulsive  attack,  one  must 
be  able  to  base  his  opinion  upon  a  very  accurate  observation  of  the  course 
of  the  disease,  the  absence  of  symptoms  of  a  cerebral  disease  focus  and  upon 
a  careful  study  of  the  history.  The  occurrence  even  of  a  unilateral  status  epi- 
lepticus  has  been  pointed  out  by  Midler. 

As  to  the  frequency  of  fits  in  epileptics,  no  general  statements  can  be 
made.  They  may  occur  very  rarely  (once  or  twice  a  year),  or  very  fre- 
quently (many  times  a  day),  and  between  these  two  extremes  there  lie  all 
possible  degrees  of  frequency.  In  women  the  menstrual  type,  in  which  the 
paroxyms  always  fall  in  the  menstrual  or  in  the  pre-  or  post-menstrual  period, 
are  relatively  frequent.  When  in  the  most  severe  cases  of  epilepsy,  one  attack 
immediately  follows  another  without  the  patient  regaining  consciousness  be- 
tween times,  this  constitutes  the  so-called  "status  epilepticus,"  a  form  of  seizure 
which  may  last  for  hours  and  is  always  very  dangerous  to  life.  The  body 
temperature  usually  increases  with  each  successive  attack,  so  that  finally 
hyperpyretic  temperatures — over  41°  C.  (106°  F.)  can  be  reached.  Also 
the  frequency  of  the  pulse  increases  in  a  threatening  manner  and  the  action 
of  the  heart  becomes  weaker.  When  status  epilepticus  is  going  to  terminate 
favorably  there  is  gradually  a  longer  interval  between  the  attacks,  the  con- 
vulsions decrease  in  severity,  the  pupils  begin  to  react  again,  the  temperature 
falls,  and  finally,  the  patient  regains  consciousness.  In  cases  terminating 
fatally  on  the  other  hand,  there  is  added  to  the  convulsions  a  condition  of 
profound  collapse  in  which  the  pupils  remain  mydriatic  and  rigid,  all  reflexes 
disappear,  breathing  becomes  continually  more  superficial,  the  pulse  indeed, 
slower  again  but  steadily  smaller,  and  finally  cessation  of  heart  action  and 
respiration  occurs. 

In  the  clinical  estimation  of  epileptic  attacks,  besides  their  frequency,  the 
time  of  day  at  which  the  paroxysms  occurs  is  important.  There  are,  besides 
patients  who  are'  only  attacked  by  their  convulsions  while  awake'  or  indiffer 
entlv  by  dav  or  by  night,  alse)  those  in  whom  exclusively  nocturnal  attacks 
occur.  Such  attacks  can  be'  easily  overlooked  when  the'  patient  sleeps  alone; 
Wetting  tin'  heel  iii  grown  people,  contusions  from  striking  the'  head  against 
the'  wall  or  on  the'  bedstead,  alse>  awakening  with  a  feeling  of  being  exhausted 
and  very  weak,  should  always  arouse'  a  suspicion  e>f  nocturnal  epilepsy.  The 
differential  diagnosis  between 'the  epileptic  and  the  hysterical  seizure  we'  will 
defer  until   Lecture  XXIX. 


392  LECTURE    XXVI 

2.     Minor  Epileptic  Attacks 
("Petit  mal,"   Epilepsia  Minor,   Non-Convulsiva) 

At  the  side  of  the  classical  epileptic  attack  just  sketched,  to  whose  most 
striking  criteria  the  convulsions  belong,  there  are  now  to  be  placed  other 
forms  of  seizure  which  with  equal  certainty  declare  themselves  manifestations 
of  the  morbus  sacer,  in  which,  however,  the  convulsive  factor  is  either  entirely 
absent  or  only  indicated.  Common  to  all,  is  the  paroxysmally  appearing,  more 
or  less  marked  clouding  of  consciousness,  which  can  increase  to  complete  loss 
of  consciousness.  In  general,  however,  their  symptomatology  varies  within 
rather  wide  limits.  We  combine  them  under  the  name  of  "minor"  or  "non- 
convulsive  epilepsy"  and  place  them  as  "petit  mal"  in  opposition  to  "haut 
mal."  Many  epileptics  suffer  from  both  major  and  minor  seizures,  while  in 
others  all  the  paroxysms  belong  to  the  same  type.  Let  us  now  enumerate  the 
most  important  varieties  of  non-convulsive  epilepsy,  proceeding  from  the  more 
frequent  to  the  less  frequent. 

A.     THE  "MOMENTARY  ARSENCE" 

This  peculiar  phenomenon  may  be  defined  briefly  as  a  sudden  and  tran- 
sitory elimination  of  the  higher  psychical  mechanisms.  In  the  simplest  cases 
the  patient  ceases  suddenly  the  work  which  is  occupying  him,  or  in  the  mid- 
dle of  a  sentence  which  he  is  just  speaking,  remains  quiet  a  few  seconds  with 
fixed  look  ("as  if  absent"),  then  resumes  again  his  occupation  or  his  con- 
versation as  if  nothing  special  had  happened,  and  with  complete  amnesia  for 
the  interruption  which  has  occurred,  exactly  at  the  point  where  the  "absence" 
began.  Usually,  however,  this  time  is  filled  out  with  peculiar  automatisms 
varying  exceedingly  from  case  to  case.  The  patient,  for  example,  emits 
grunting  or  snoring  noises,  makes  movements  of  chewing,  a  few  twitching 
movements  of  the  face  or  of  the  extremities,  claps  his  hands,  bends  himself 
forward  as  in  "salaam  spasm"  (see  above,  page  69),  speaks  senseless  words 
("epilepsie  marmottante"),  etc.  Rarely  the  automatisms  assume  a  more 
complicated  form,  as  was  the  case  in  the  epileptic  President  of  the  Court 
described  by  Trousseau,  who  in  the  middle  of  a  session,  rose,  muttered  some- 
thing unintelligible,  went  from  the  Tribunal  into  the  robing-room,  there 
urinated  on  the  floor,  then  returned  to  his  post  and  took  up  again  the  pro- 
ceedings where  he  had  broken  off.  Occasionally  during  the  absence,  the  occu- 
pation is  not  really  interrupted,  but  is  carried  on  in  a  senseless,  purely  me- 
chanical manner,  so,  one  of  my  patients,  a  dactylographer,  became  aware  of 
her  petit  mal  attacks,  by  finding  that  in  the  middle  of  the  text  which  she 
was  copying,  there  was  a  half  line  of  letters  placed  together  indiscriminately. 

R.     EPILEPTIC    VERTIGO 

In  epileptic  vertigo  a  feeling  of  turning  or  of  loss  of  equilibrium  suddenly 
comes  over  the  patient ;  he  totters,  but,  as  a  rule,  before  falling,  regains  his 
equilibrium.     These  attacks  are  usually  accompanied  by  pallor,  sometimes  also 


EPILEPSY  393 

by  the  escape  of  a  few  drops  of  urine ;  consciousness  is  practically  never  com- 
pletely lost,  although  it  is  markedly  clouded  (a  transitory  feeling'  of  con- 
fusion, of  defective  psychic  orientation).  Epileptic  vertigo  is  hence  consid- 
ered as  an  "attenuated  attack"  of  the  morbus  sacer. 

C.     EPILEPTIC  SYNCOPE 

Epileptic  syncope  can  be  considered  as  a  higher  degree  of  intensity  of 
epileptic  vertigo,  since  it  begins,  as  a  rule,  with  the  feeling  of  apparent  move- 
ment, tottering,  etc.,  still,  here  the  patient  has  loss  of  consciousness  and  ac- 
tually falls,  hut  regains  consciousness  in  a  short  time.  Spasms  are  absent 
or  only  slight.     Such  attacks  have  been  also  denominated  "apoplectiform." 

D.     THE  "NARCOLEPTIC"  ATTACK 

In  this  there  is  sudden  falling  asleep  in  the  daytime  and  the  patient  cannot 
be  awakened  by  shaking,  etc.  After  spontaneous  awakening  the  patient  often 
shows  a  delirious  condition;  in  any  case,  however,  he  has  no  consciousness  of 
having  been  asleep. 

E.     THE   "PROCURSIVE   EPILEPTIC"   ATTACK 

In  the  paroxysms  which  have  been  denominated  "procursive  epilepsy" 
(Boufneville  and  Ladame)  there  is  a  sudden  running  forward  in  which  the 
patient  either  avoids  objects  which  stand  in  his  way  or  forcibly  pushes  them 
aside.  Here  also  there  is  lack  of  memory  for  the  attack,  sometimes  also  for 
the  period  immediately  preceding  it.  A  still  more  rare  variety  is  the  "retro- 
pulsive  epilepsy"  of  Launois,  in  which  the  patient  in  his  attacks  runs  backward. 

3.     Epileptic   Equivalents 

As  equivalents  in  the  broad  sense  we  denominate  manifold  transitory  phe- 
nomena, which  in  the  concrete  ease  we  consider  as  of  epileptic  nature,  since 
they  appear  to  occur  in  epileptic  individuals,  or  members  of  epileptic  families, 
in  the  place  of  the  major  or  minor  attacks  propel-,  run  their  course  usually 
with  more  or  less  clouded  consciousness,  are  generally  followed  by  a  condition 
of  decided  prostration,  and  often  react  in  a  striking  way  to  bromide  treat- 
ment (see  below).  For  example,  outbreaks  of  sweat,  trigeminus  neuralgia, 
profuse  di.i rrhcea  ^,  Quincke's  oedema  (see  Lecture  XXV),  sialorrhoea,  vomiting, 
attacks  of  migraine,  stenocardic  attacks,  paroxysmal  tachycardia,  spasm  of 
Hie  glottis,  .tc.  .Much  more  important,  however,  bhan  these  sensory,  vaso 
motor  and  viscera]  equivalents  (whose  inclusion  within  the  limits  of  epilepsy 
is  through  quite  a  wide  extension  of  hypothesis),  .ire  the  psychical,  for  which 
in  general  w<  reserve  Hie  term  "equivalent"  in  tin-  narrower  sense.  Falret  lias 
divided  them  according  to  the  intensity  of  their  symptoms  into  "intellectual 
grand  mal"  and  "intellectual  petit  mal,"  a  distinction  which  i>  ton  arbitrary 
to  have  been   generally   received. 

There    occur,    for    example,    in    attacks,    hallucinations    with    conditions    of 


394  LECTURE    XXVI 

great  anxiety,  maniacal  deliria,  which  under  the  influence  of  delusions  and 
hallucinations  can  increase  to  frenzy  and  destructive  rage,  particularly,  how- 
ever, impulsive  acts  of  sometimes  quite  complicated  content.  In  these  differ- 
ent manifestations  the  patients  are  in  a  condition  of  abnormal,  dreamy  con- 
sciousness ("dammerzustand"  "etat  second")  for  which  after  the  paroxysm 
has  passed  off,  memory  is  by  no  means  always  obliterated  (forensically  im- 
portant), almost  always,  however,  markedly  cloudy.  The  impulsive  equiva- 
lents of  epileptics  as  well  as  epileptic  mania,  are  responsible  for  many  mis- 
demeanors and  crimes.  Here  belong  cases  of  incendiarism,  motiveless  homi- 
cides, thefts  in  part  miscellaneous,  in  part  confined  to  certain  things,  often 
openly  carried  out,  public  exposure  of  the  genitals  (exhibitionism),  etc.  Upon 
an  epileptic  basis,  dipsomanic  attacks  may  also  rest, — sudden  impulsion  to 
senseless  drinking  themselves  drunk,  in  previously  abstemious  persons.  These 
equivalents  are,  on  the  average,  of  considerably  longer  duration  than  epileptic 
attacks  proper;  the  abnormal  condition  can  last  for  hours,  even  days.  The 
last  is  the  rule  in  the  interesting  phenomenon  which  has  been  denominated 
"ambulatory  automatism,"  "poriamania,"  or  epileptic  "wandertrieb."  The 
patient  suddenly  leaves  without  motive  his  place  of  residence,  and  wanders 
blindly  about,  during  which  time  as  far  as  buying  tickets,  paying  his  way, 
etc.,  is  concerned,  he  may  conduct  himself  in  an  entirely  unobtrusive  and 
normal  manner.  When  the  equivalent  passes  away,  either  there  is  no  recollec- 
tion of  the  clouding  of  consciousness,  or  the  patient  has  the  feeling  of  waking 
from  a  dream  whose  content  he  but  dimly  remembers.  A  patient  of  Legrand 
du  Soulle  gained  fame  from  having  been  overcome  by  his  poriomania  in  Havre, 
and  having  to  his  boundless  astonishment  come  to  himself  again  in  Bombay. 
The  epileptic  clouding  of  consciousness  ("dammerzustand")  as  it  serves 
as  the  basis  of  different  psychical  equivalents,  can  appear  as  the  forerunner 
of  a  convulsive  attack,  or  in  connection  with  such  an  attack.  In  the  cases 
in  which  it  does  not  occur  alone  in  the  place  of  a  seizure,  we  speak  of  pre- 
epileptic or  post-epileptic  clouding  of  consciousness  (delirium),  ("prepar- 
oxysmal"  and  "postparoxysmal"  or  "preconvulsive"  and  "postconvulsive" 
would  be  more  correct). 

4.      The    Interparoxysmal    Anomalies 

When  we,  as  is  usually  the  case  in  the  ambulatory  treatment,  examine  epi- 
leptics outside  of  their  attacks,  we  find  occasionally  an  entirely  normal  status 
and  must  base  our  estimation  of  the  case  entirely  upon  the  history.  Neverthe- 
less, a  thorough  investigation  in  the  majority  of  cases  discloses  definite  anom- 
alies either  in  the  bodily  or  in  the  mental  sphere. 

Under  the  first  we  may  mention  at  the  start  the  so-called  stigmata  of 
degeneration  which  many  epileptics  (as  many  constitutional  neuropaths  in 
general)  present.  Most  frequent  are  anomalies  of  the  skull  of  the  most  varied 
sort,  for  example,  marked  asymmetry,  abnormal  shape  of  the  palate  (Gothic 
palate),  abnormalities  of  the  teeth,  microcephaly,  etc.  Further,  malforma-' 
tions  of  the  ear  (adhesion  of  the  lobe,  faun-like  pointed  or  protruding,  the 
so-called  "jug  handle"  ears,  etc.),  and  congenital  anomalies  of  the  eye  (colo- 


EPILEPSY  395 

boma  iridis,  marked  astigmatism,  etc.)-  Much  rarer  are  signs  of  degenera- 
tion on  the  trunk  and  on  the  extremities,  as,  for  example,  funnel  chest,  syn- 
dactylism, melanodermia.  In  the  second  place  I  would  relate  the  muscular 
anomalies,  above  everything  the  muscular  weakness  appearing  in  contrast  to 
the  good  morphological  development  of  the  muscles,  which  relatively  often 
appears  upon  one  side.  Less  frequent  are  muscle  disturbances  of  atrophic 
character  to  which  Onufrowicz  has  called  attention.  So,  the  occasionally  ob- 
served scapula  alata  is  to  be  referred  to  a  paresis  of  the  serratus  magnus  which 
may  be  accompanied  by  that  of  the  trapezius,  the  rhomboids  and  the  levator 
scapula.  That  infantile  cerebral  palsy  is  often  combined  with  epilepsy  we 
have  already  emphasized  in  Lecture  XXII.  Also  myoclonus  epilepsy  has  al- 
ready been  considered  (see  page  73).  Almost  all  epileptics  show  an  unusual 
activity  of  the  tendon  reflexes,  many  are  left-handed  or  ambidextrous. 

We  find  further,  occasionally,  general  reduction  of  the  superficial  sensibility 
as  well  as  the  most  varied  speech  defects.  Finally,  the  scars  of  tongue-bites 
and  other  wounds  occurring  in  the  attacks  should  be  considered. 

Practically  much  more  important,  are,  however,  the  (often  progressively 
appearing)  permanent  psychic  and  character  alterations  of  epileptics.  We 
will  leave  out  of  consideration  here  those  cases  in  which  the  combination  of 
congenital  idiocy  with  epilepsy  is  present  (see  Lecture  XXII,  page  336)  and 
confine  ourselves  to  the  consideration  of  such  anomalies  which  must  be  inter- 
preted as  the  result  cf  epilepsy,  not  as  a  pathological  condition  coordinate 
with  this  last.  Many  epileptics  are  excessively  quick-tempered  and  inclined  to 
violence  and  sudden  changes  of  mood,  malignant,  cruel,  sexually  perverse,  of 
brutal  egotism.  In  severe  cases,  on  the  other  hand,  there  appears,  little  by 
little,  a  continually  plainer  reduction  of  the  judgment-forming  capacity  which 
increases  to  actual  dementia.  In  the  severest  cases  the  dementia  may  be  com- 
plete with  loss  of  all  mental  capacity  and  even  of  speech,  uncleanness,  refusal 
of  food,  etc.  "Epileptic  paranoia,"  in  which  ideas  of  unseen  Influence  and  de- 
lusions of  persecution,  on  account  of  the  violence  of  character  of  epileptics, 
may  be  particularly  dangerous,  i>  rare.  To  these  psychical  disturbances 
peculiar  to  epilepsy  are  opposed  the  numerous  milder  cases  in  which  character 
and  intelligence  remain  normal  during  life.  It  stands  beyond  question  that 
many  eminent  personages  have  been  epileptics.  I  myself  know  undoubted  cases 
of  this  sort  from  my  own  practice.  It  must,  indeed,  be  said  that  in  the  so- 
called  "pathographies"  with  retrospective  diagnosis,  epilepsy  has  often  been 
diagnosed  with  unbelievable  carelessness,  for  example,  in  relation  to  Napoleon 
I.  That  he  at  the  end  of  the  battle  of  Wagram  at  a  moment  when  the  pursuit 
of  the  enemy  still  imposed  upon  him  important  duties,  suddenly  lay  down  on 
the  floor  and  fell  into  a  deep  sleep,  need  not  be  interpreted  as  narcolepsy, 
win  n  one  remembers  that  for  over  bS  hours  in  spite  of  the  greatest  mental 
tension   and   bodily   exertion,  he  had   not    slept   one  second. 

Etiology  and  Pathological  Anatomy 

Gentlemen:  That  heredity  plays  >  particularly  greal  rfile  in  the  patho- 
genesis of  the  disease  wluhr   symptomatology    I   have   now  brought    before  you, 


390  LECTURE    XXVI 

there  can  be  no  doubt.  Binswanger  has,  for  example,  been  able  to  recognize 
mental  and  nervous  diseases  among  the  ascendants  of  from  35  to  40  per  cent, 
of  his  epileptics,  and  the  heredity  can  be  equally  like  or  unlike.  Just  as  gen- 
erally recognized  is  the  frequency  of  epilepsy  among  the  descendants  of 
drinkers,  which  we  can  interpret  in  the  sense  of  injury  to  the  germ  ("blastoph- 
thoria")  through  parental  alcoholism  (compare  Lecture  VIII,  page  127). 
Further,  numerous  observations  speak  for  the  fact  that  the  changes  in  the 
brain  which  form  the  basis  for  genuine  epilepsy  can  be  produced,  or  at  least 
favored,  by  exogenic  factors.  This  applies  particularly  in  childhood ;  that 
"late  epilepsy"  is  so  rare  depends  upon  the  fact  that  after  its  development 
is  completed,  the  brain  has  much  less  tendency  to  react  to  pathological  altera- 
tions by  paroxysmal  discharges.  Among  the  acute  infectious  diseases  in  direct 
sequence  to  which,  or  during  whose  course  the  outbreak  of  epilepsy  has  often 
been  observed,  scarlatina  takes  readily  the  first  place;  next  to  it  are  to  be 
mentioned  particularly  small-pox,  influenza,  measles  and  typhoid  fever. 
Among  the  chronic  infectious  diseases  hereditary  syphilis  occupies  the  first 
place.  For  late  epilepsy,  acquired  syphilis  comes  into  consideration.  Here, 
however,  certain  intoxications  appear  very  frequently  in  the  etiology:  Saturn- 
ism, cocainism,  alcoholism  (when  we  speak  of  the  latter  we  must  make  it  clear 
to  ourselves  whether  or  not  it  is  a  dipsomanic  form  which  is  to  be  considered 
as  a  symptom  of  epilepsy,  not  its  cause).  Circulatory  disturbances  also  may 
play  a  role  in  the  etiology  of  late  epilepsy-  Often  its  beginning  coincides 
with  tlie  development  of  severe  arteriosclerosis;  here,  also,  belong  the  not  rare 
observations  of  epileptic  attacks  in  diseases  of  the  heart  and  aorta.  Finally, 
it  is  to  be  considered  as  proved  that  injuries  to  the  head  can  lead  to  genuine 
epilepsy  (not  only  to  the  Jacksonian  form  depending  upon  a  circumscribed 
lesion). 

A  special  position  in  the  discussion  of  epilepsy  is  assumed  by  the  so-called 
"reflex  epilepsy,"  whose  origin  still  remains  a  riddle.  Sometimes  in  previously 
healthy  persons  after  wounds  of  the  extremities,  of  the  trunk,  or  of  the  head, 
there  appear  typical  epileptic  attacks  which  are  introduced  by  a  sensory  aura 
starting  in  the  scar  left  behind  by  the  trauma.  Sometimes  indeed,  an  attack 
may  be  produced  by  pressure  upon  such  a  scar  (which  then  is  denominated  an 
"epileptogenic  zone").  Occasionally  diseases  of  the  uterus,  of  the  nose  and  its 
accessory  cavities,  etc.,  can  act  in  a  similar  manner,  while  the  convulsions  of 
children  having  intestinal  parasites  do  not  usually  appear  to  be  of  epileptic 
character.  Further,  many  cases  of  hysterical  attacks  have  been  incorrectly 
considered  as  reflex  epilepsy.  Nevertheless,  there  remains  a  respectable  num- 
ber of  these  cases  which  have  been  regarded  as  truly  epileptic  by  recognized 
authorities,  as  for  example,  Oppcnlu'im.  On  account  of  the  great  rarity  of 
such  true  reflex  epilepsy  in  spite  of  the  frequency  of  wounds,  we  must  assume 
that  in  the  individuals  affected,  the  latent  predisposition  to  epilepsy  is  anatom- 
ically predetermined  and  the  irritation  from  the  scar,  etc.,  only  furnishes  the 
exciting  cause.  Excision  of  the  scar  or  removal  of  any  other  pathological 
condition  frequentty  does  away  with  the  reflex  epileptic  attacks. 

Let  us  now  pass  on  to  the  pathological  anatomy  of  the  morbus  sacer. 
You  have  remarked  that  I  have  repeatedly  spoken  of  a  brain  disease,  of  anatom- 


EPILEPSY  397 

ical  alterations,  etc.  There  is  for  me  to-day,  as  for  the  great  majority  of 
neurologists,  no  longer  the  slightest  doubt  that  material  alterations  of  the 
cerebral  cortex  lie  at  the  basis  of  epilepsy,  though  they  are  here  and  there 
too  little  intense  to  be  discovered  by  our  histological  methods.  Epilepsy  is 
no  "neurosis,"  and  Hippocrates  has  rightly  remarked  that  its  etiology  and 
pathology  are  in  the  brain. 

Chaslin,  in  1889,  published  the  first  findings,  but  in  late  years  these  have 
been  extended  and  systematized  by  Alzheimer  through  finer  methods.  In 
many  cases  of  genuine  epilepsy  he  has  shown  a  "marginal  gliosis"  of  the  cere- 
bral cortex  with  numerical  reduction  of  the  medullated  fibres  and  ganglion, 
cells ;  the  latter,  besides  this,  appear  atrophic.  The  vessels  are  proliferated, 
show  thickened  walls,  sometimes  also  lymph  sheaths  filled  with  mast  cells.  In 
patients  who  have  died  in  attacks  (or  in  status  epilepticus)  there  are,  besides 
severe  acute  changes  in  the  ganglion  cells  and  their  axis  cylinders,  also  ame- 
boid glia  cells  loaded  with  products  of  disintegration.  The  last  mentioned 
anomalies  indeed  are  connected  with  the  epileptic  attacks,  while  the  marginal 
gliosis  and  its  accompanying  changes  can  be  held  responsible  for  the  inter- 
paroxysmal  clinical  pictures,  particularly  for  the  dementia  and  change  of 
character.  On  the  other  hand,  Alzheimer  attributes  no  great  importance  to 
th'-  sclerosis  of  the  cornu  Ammonis  present  in  50  to  60  per  cent,  of  these 
cases,  considers  it  rather  only  as  an  occurrence  associated  with  the  epileptic 
degeneration. 

What,  however,  causes  the  intermittent,  sometimes  even  explosive,  onset 
of  convulsive  or  other  paroxysmal  phenomena?  Where  peripheral  irritative 
conditions  cannot  come  into  question  as  in  reflex  epilepsy,  it  is  natural  to 
consider  autotoxic  factors  and  many  authors  have  instituted  investigations 
in  this  direction,  into  which,  since  they  concern  things  still  quite  hypothetical, 
«!■  cannot  inter  further.  We  need  only  mention  as  an  example,  the  work  of 
Donath,  who  asked  himself  what  substances  introduced  into  the  circulation  of 
animals  are  able  to  set  off  epileptic  paroxysms.  lie  has  come  to  the  conclu- 
sion that  the  organic  bases  resembling  ammonia  ( triincthvlamin,  cholin,  ere 
atinin.  guanidin  )  act  as  eonvulsants.  He  hence  believes  that  in  epileptics  the 
occasional  accumulation  of  substances  of  this  sort  in  the  blood  irritate  the 
morbidly  constituted  brain  to  react  by  epileptic  attacks.  That  the  blood 
of  epileptics  during  the  attack  possesses  toxic  properties  appears  probable 
from  the  investigations  of  Cent  and  others,  without  our  knowing  how  to  make 
much  use  of  this  pathogenetic  fact. 

,      Prognosis 

Epileptic  attacks  usually  only  indirectly  endanger  the  life  of  the  patient, 
that  is,  in  consequence  of  the  injuries  which  he  can  sustain  by  falling:  as 
already  said,  the  status  epilepticus,  whose  prognosis  in  the  individual  case 
I  have  already  discussed,  furnishes  an  except  ion  to  this.     Though  epilepsy  in 

itself  does  not  directly  put  life  into  question,  still,  patients  with  the  severe 
form  seldom  live  to  he  old.  since  they  USUallj  have,  in  general,  a  reduced  in- 
sistence   to    all    possible    injurious    factors.       To    definite    recovery    at    mo-t     10 


398  LECTURE    XXVI 

to  15  per  cent,  of  the  cases  are  to  be  brought  by  proper  treatment,  still  there 
are  along  with  these,  cases  in  which  a  very  great  improvement  is  to  be  ob- 
tained, so  that,  for  example,  the  attacks  only  show  themselves  occasionally, 
at  intervals  of  a  year  or  more.  The  earlier  the  disease  begins  and  the  more 
frequent  the  attacks,  the  worse  the  prognosis  as  to  recovery  and  also  as  to 
mental  integrity.  On  the  other  hand,  the  occurrence  of  major  attacks  does  not 
at  all  cloud  the  prognosis,  in  contradistinction  to  the  forms  manifesting  them- 
selves in  minor  attacks  and  equivalents.  On  the  contrary,  the  last  are  much 
more  difficult  to  influence  therapeutically  and  in  the  long  run  never  leave  the 
psyche  intact.  Patients  with  "procursive  epilepsy"  usually  become  demented 
particularly  rapidly. 

T  rentmcnt 

In  the  foreground  of  the  treatment  of  epilepsy  in  spite  of  the  manifold 
other  methods  of  medication  continually  appearing  (most  of  which  fall  again 
into  oblivion),  stands  always  the  intelligent  application  of  the  alkaline  bro- 
mides which  we  will  hence  discuss  with  a  thoroughness  corresponding  to  its 
importance. 

In  the  following  I  will  not  consider  the  severe  cases  of  epilepsy  or  those 
combined  with  psychoses  or  idiocy,  needing  asylum  care,  but  will  keep  in  view 
those  suitable  for  ambulant  treatment  or  management  at  home.  Patients 
entering  upon  treatment  are  particularly  to  be  impressed  with  the  fact,  that 
under  all  circumstances  the  cure  must  extend  long  after  the  cessation  of  at- 
tacks, also  that  they  must  not  interrupt  it  even  for  a  few  days  of  their  own 
accord ;  women,  also  that  bromide  medication  is  in  no  way  incompatible  with 
menstruation  and  pregnancy.  Some  person  connected  with  the  patient  must 
assume  responsibility  for  the  strict  carrying  out  of  orders  and  must  keep  an 
accurate  account  of  the  number,  duration  and  severity  of  the  attacks. 

Among  the  alkaline  bromides,  potassium  bromide  is  undoubtedly  the  most 
efficient  preparation,  probably  because  it  is  more  slowly  excreted  than  sodium 
bromide  and  ammonium  bromide  (the  last  is  in  spite  of  its  high  content  of 
bromine,  of  exceedingly  low  activity;  strontium  bromide  has  almost  only  his- 
torical interest).  Unfortunately  the  disagreeable  associated  effects  of  bromide 
medication  are  much  more  frequent  and  intense  with  potassium  bromide  than 
with  the  other  bromine  preparations  (bromide  acne,  gastro-intestinal  disturb- 
ances, general  prostration,  etc.),  which  sometimes  moves  us  to  abandon  the 
potassium  for  the  sodium  salt  or  for  "Erlenmeyer's  Mixture" — 2  parts  each 
of  KBr  and  NaBr,  1  part  of  AmBr.  The  "injurious  action  of  potassium 
upon  the  heart  muscle"  of  which  we  still  hear  occasionally,  need  not  be  brought 
up  against  potassium  bromide,  since  it  is  never  observed  in  practice.  To 
handle  a  case  of  genuine  epilepsy  with  organic  bromine  preparations  is  an 
attempt  which  you  will  always  regret.  Next  to  the  choice  of  a  pi'eparation, 
its  dosage  is  of  great  importance.  Often  there  is  the  tendency  to  make  it  too 
low  at  the  start.  In  general,  one  should  not  begin  with  less  than  2.0  KBr 
a  day.  Giving  a  full  day's  dose  at  one  time  (before  retiring)  is  the  rule  in 
nocturnal  epilepsy;  in  diurnal  forms,  however,  or  in  those  which  do  not  occur 
at  any  definite  time  of  day,  you  will  generally  do  better  (though  not  always) 


EPILEPSY  399 

if  you  divide  the  day's  supply  into  2  doses  of  1.5  each.  The  patient  must 
take  one  at  bed-time  and  one  at  breakfast-time,  or — when  he  tends  to  have 
attacks  immediately  after  rising — half  an  hour  before  leaving  his  bed.  If 
this  dose  proves  insufficient  we  add  1.0  to  1.5  KBr  at  mid-day.  Casus  in  which 
daily  doses  of  from  -1.0  to  5.0  show  no  effect,  for  further  increase  of  the  dose 
(to  6.0  or  8.0  and  more)  are  best  brought  under  hospital  treatment  or  into 
special  institutions  for  epileptics.  Epileptics  are  almost  always  more  tolerant 
of  bromides  than  other  individuals;  particularly  so  are  epileptic  children. 
Since  my  remarks  up  to  this  time — as  also  the  recipes  which  I  will  give  you 
later — are  adapted  to  adults,  you  may  note  the  following  figures  as  represent- 
ing the  usual  doses  of  potassium  bromide  for  young  epileptics  of  slight  degree: 

Single  Dose.  Dose  per  Day. 

Two  first  years  of  life 0.1    (gr.  1%)  0.3    (gr.  4>V2) 

3d     to     5th  year 0.2   (gr.  3)"  0.5    (gr.  Tl/o) 

6th   to    11th   year 0.5   (gr.  7V2)  1.0   (gr.  15) 

12th   to    16th   year 0.5    (gr.  71  2)  2.0   (gr.  30) 

Still,  when  necessary,  children  can  bear  much  higher  doses  very  well,  for 
example,  2.5  per  day  in  the  fourth  year. 

After  the  patient  has  become  accustomed  to  a  plainly  efficacious  initial 
dose  (for  this  several  weeks  are  needed),  for  his  further  management,  there  is 
a  choice  between  the  following  methods: 

(«)  The  method  of  increasing  and  decreasing  doses  (Charcot).  This  is 
especially  suited  for  cases  with  paroxysms  appearing  more  or  less  periodically, 
for  example,  for  the  menstrual  or  pre-menstrual  attacks.  During  the  week 
in  which  attacks  of  the  last  kind  are  to  be  expected  the  patient  receives  the 
day's  dose  proved  to  be  efficient  (let  us  say  1.0 );  during  the  following  week, 
3.0  a  day:  the  next  week,  2.0;  then  for  one  week  again  3.0,  and  finally  at  the 
critical  period,  again  1.0.  After  several  months  we  try  if  3.5  or  3.0  is  not 
a  sufficient  maximum  dose,  and  if  the  result  is  obtained,  in  the  course  of  several 
years  continually  raising  and  lowering  the  dose,  we  endeavor  to  dispense  witli 
the  bromide  allogetlii  r. 

(?;)  The  method  of  gradual  disuse.  This  is  particularly  suited  for  cases 
with  irregular,  entirely  unaccountable  attacks  and  those  in  which  the  seizures 
rapidly  follow  one  another.  The  ascertained  efficient  dose  is  continued  for 
months,  then  it  is  attempted  every  few  weeks  t,,  reduce  it  about  0.5  a  day. 
If  the  case  gels  worse  we  return  to  the  next  higher  dose  and  endeavor  to 
reduce  it  again  some  months  later.  Even  when  a  gradual  withdrawal  is  carried 
out  without  disturbance,  we  are  very  cautious  about  reducing  the  last  dose  and 

wait    until    no   attack    has   occurred    for   at    least    a   year.      Then   we    proceed    best 

after  the  method  of  Legrand  du  Saulle:  for  3  months,  <i  "bromide  days"  a 
week;  for  3  months,  5  "bromide  days";  for  3  months,   1  "bromide  days,"  etc. 

The  most  important  adjuvant  of  the  alkaline  bromide  therapy,  is  the  -.ill 

lie,'    Hi-    salt-poor   diet    after    ToilloUSl     and    li'itlitt.       Kemo\al    or    reduction    of 

the  chloride  of  sodium  in  the  did  increases  the  pharmacodynamic  action  of  the 
alkaline  bromides   so  greatly,   that    we   can   almost    always   get    along  with   much 


400  LECTURE    XXVI 

smaller  doses  and  can  succeed  sometimes  with  3.0  to  4.0  KBr  in  a  severe,  and 
even  2.0  in  a  moderately  severe  epilepsy.  Unfortunately  there  are  plainly  indi- 
vidual differences,  and  here  and  there  no  special  result  can  be  obtained  with  the 
T oulousc-Richet  method.  Since  this  last  not  only  increases  the  efficacy  of  the 
bromide  action,  but  also  the  danger  of  bromism,  in  practice,  only  the  relative 
removal  of  salt  comes  into  consideration  (for  example,  ordinary  but  unsalted 
food  with  500  grin  of  salted  bread=2.5  NaCl).  The  technique  of  a  salt-poor 
diet  is  considerably  simplified  by  the  "Sedobrol"  tablets  proposed  by  Ulrich: 
these  contain  1.5  bromide  of  sodium  and  0.1  grm  chloride  of  sodium,  with  fat 
and  extractives  of  vegetable  albumen  as  a  seasoning.  Dissolved  in  hot  water 
they  give,  depending  upon  concentration,  an  almost  salt-free,  palatable  soup 
or  a  sauce  very  suitable  for  salt-free  vegetable  food.  As  to  the  rest  of  the 
food,  a  predominantly,  but  not  exclusively  lacto-vegetarian  diet  is  to  be  recom- 
mended. On  the  other  hand,  strongly  spiced  foods  are  to  be  avoided.  Alcoholic 
beverages  are  to  be  absolutely  forbidden ;  coffee,  tea  and  tobacco  are  to  be 
permitted  only  in  very  small  quantities.  Conditions  of  life  as  quiet  as  possible 
are  to  be  provided  for. 

If  bromide  acne  appears,  compresses  of  from  10  to  20  per  cent,  salt  solu- 
tion applied  to  the  diseased  portions  of  the  skin  act  very  favorably,  as  Ulrich 
has  shown  ;  of  internal  medications,  Fowler's  solution  acts  best.  Upon  the  large 
acne  nodules,  pieces  of  mercurial  plaster  are  placed. 

The  addition  of  sodium  benzoate  (G.  dc  la  Tourctte)  or  Adonis  vernalis 
(Bechterew)  appears  to  increase  the  efficacy  of  the  alkaline  bromides;  the  first, 
perhaps,  by  aiding  the  absorption  from  the  intestine,  the  last  by  improving  the 
brain  circulation ;  both  drugs  act  as  diuretics  opposing  brominism.  For  severe 
cases,  the  combination  of  bromide  and  chloral  hydrate,  which  in  evening  doses 
of  from  0.3  to  1.0  can  be  given  for  a  long  time  without  any  hesitation,  has 
proved  particularly  satisfactory.  Valerian  also  aids  the  action  of  the  bromide 
somewhat.  On  the  other  hand  I  would  imperatively  warn  you  against  the  ap- 
plication of  "Flcchig's  Bromide-Opium  Cure"  outside  of  a  hospital  This 
method,  in  which  the  patient  within  6  weeks  is  brought  to  take  0.9  extract, 
thebaicum  per  day,  upon  the  substitution  for  which  of  bromide  of  potassium 
this  last  works  particularly  intensely,  has  caused  some  deaths. 

Still  a  few  words  upon  the  prescribing  of  alkaline  bromides  in  epilepsy. 
For  poor  people  the  bromide  of  potassium  can  be  prescribed  as  the  salt,  from 
which  the  patient  prepares  for  himself  a  solution  of  definite  concentration.  In 
general  it  is  convenient  to  prepare  solutions  of  such  strength  that  a  table- 
spoonful  (or  a  measuring-glass  of  15  cc)  or  a  teaspoonful  (5  cc)  corresponds 
to  1.0  bromide  of  potassium.  The  solutions,  on  account  of  the  effect  on  the 
stomach,  should  always  be  taken  much  diluted.  On  account  of  the  long  duration 
of  the  cure,  the  solutions  of  the  alkaline  bromides  or  the  salts  are  prescribed  in 
large  quantities  (as  long  as  substances  which  do  not  keep  well  are  not  mixed 
with  them).  It  only  remains  for  me  to  give  you  the  formula?  for  several  com- 
binations of  the  alkaline  bromides  which  have  proved  useful. 


EPILEPSY  401 

I>      Potassi  bromid 200.0 

Infus.  radic  valer.  (20  per  cent.)  ad.    1000.0 

M.  S.     One  teaspoonful=1.0  grin.  KBr. 

Ifs      Potassi  bromide 40.0   ( 10  drachms) 

Sodii  bromide, 
Amnion,   bromide, 

Sodium  benzoat    aa         12.0   (3  drachms) 

Aq.    mentli.    pip 1000.0    (1   quart) 

M.  S.     One  tablespoonful=1.0  grm.  bromides.     (15  grains) 

3^      Potassi  bromide, 

Sodium  bromide   aa.      100.0   (3  oz.) 

Amnion,  bromide    50.0    (l*--.  oz-) 

M.  S.     Keep  in  a  well-stoppered  bottle. 
X  grammes  dissolved  in  water  2  to  3  times  a  da  v.* 

1$      Potassi  bromide 20.0   (5   drachms) 

C'odeini   phosphor 0.2    (3  grains) 

1%  Inf.  Adonidis  vernalis  q.  s.  .  .  .ad.      300.0  (10   oz.) 

M.  S.     One  tablespoonful=l  gnu.  KBr.     (15  grains) 

The  individual  epileptic  attack  furnishes,  as  a  rule,  no  indication  for  thera- 
peutic interference.  Only  in  threatening  asphyxia  must  we  proceed  to  artificial 
respiration,  injections  of  ether,  camphor,  etc.  In  general  we  content  ourselves, 
however,  with  placing  the  patient  so  that  he  cannot  injure  himself  during  the 
convulsions,  loosening  his  clothing,  and  placing  a  rolled-up  handkerchief  be- 
tween his  teeth  to  prevent  him  biting  his  tongue. 

A  cutting  short  of  the  attack  through  certain  manipulations  which  they 
have  come  upon  empirically  occasionally  appears  possible  in  certain  patients. 
One  can  mainly  reckon  upon  this  possibility  in  the  so-called  "reflex  epilepsy" 
in  which,  tying  a  band  tightly  about  the  limb  wherein  the  sensory  aura  appears 
can  prevent  the  threatening  attack.  One  of  my  patients  whose  attacks  are. 
preceded  by  an  acoustic  aura,  can  prevent  it  by  inflation  of  the  Eustachian 
tubes;  in  a  patient  of  Heilbron/ner  singing  has  had  the  same  effect  ;  two  epilep- 
tics of  0 fipt  iilui m's  have,  indeed,  found  it  possible  to  effect  this  end  by  a  sim- 
ple effort  of  the  will. 

In  status  epilepticus  active  intervention  is  always  in  place.  Subcutaneous 
aseptic  injections  of  sterile  sodium  bromide  solution  can  lie  recommended. 
Dosage  (according  to  Morgan  and  Hodskins)  100  to  180  cc  of  a  (i  per  cent, 
solution:  place  of  injection,  thigh  or  back.  The  painful  induration  which  these 
injections  produce,  must  lie  treated  feu-  several  days  with  cataplasms.     Large 

Bromides  are  very  heavy.     A   teaspoonful  equals  about  8.0  grm.     In  order  l"  spare  the 

patient  weighing  nut  liis  dose  every  li lie  can  !"■  given  :>  test  tube  <»n  which  is  filed  a  line 

corresponding  In  the  point  I"  which  ii  must  be  filled  in  get  ■•<  definite  quantity  of  (he  mixed 
bromides. 


402  LECTURE    XXVI 

doses  of  bromide  of  sodium  (15.0)  can  also  be  given  in  a  clysma;  however,  the 
action  when  given  in  this  way  is  naturally  slower.  For  rectal  application 
hence,  chloral  is  better  suited  (4.0  grm  in  very  dilute  solution).  A  further, 
drug  which  sometimes  is  useful  in  status  epilepticus  is  scopolamin  hydrobromide 
in  large,  even  overmaximum  doses  (0.001  to  0.002).  Also  chloroform  nar- 
cosis is  sometimes  applied  as  a  last  resort. 

The  question  of  the  operative  treatment  of  epilepsy,  in  so  far  as  one  has 
in  view  genuine  epilepsy,  and  does  not  consider  the  results  in  such  epileptiform 
attacks  as  are  produced  by  local  circumscribed  disease  processes  (cysts,  menin- 
geal adhesions,  exostoses  of  the  skull,  etc.),  is  still  under  discussion.  Kocher 
has  expressed  himself  in  favor  of  the  undertaking  of  a  craniectomy  through 
which  beneficial  relief  of  pressure  is  to  be  effected.  Against  this  theory  of  re- 
lief of  pressure  is.  however,  to  be  opposed  the  fact  that  Tissot  for  years  has 
alternately  left  epileptic  patients  alone  or  has  subjected  them  to  regular  lum- 
bar puncture.  He  found  that  lumbar  puncture,  regularly  repeated,  even  for 
a  long  time,  exerted  a  modifying  influence  neither  upon  the  course  nor  upon, 
the  character  of  their  paroxysms.  Also  he  could  find  no  correlation  between 
the  pressure  and  the  quantity  of  spinal  fluid  on  the  one  hand,  and  the  con- 
vulsive attacks  on  the  other.  In  favor  of  partial  excisions  of  the  cortex.  .S'. 
Auerbach  has  expressed  himself  of  late:  for  "brain  massage,"  E.  Bircher.  I 
must  acknowledge  that  I  have  seen  so  many  cases  of  genuine  epilepsy  operated 
upon  without  result,  that  I  am  inclined  to  refer  the  favorable  changes  in  the 
character  of  the  disease  which  have  here  and  thei'e  been  observed  after  opera- 
tions, to  accidental  coincidence. 

On  account  of  the  dangerous  character  of  epileptics  who  have  undergone 
psychic  alteration  in  any  considerable  degree,  care  should  of  course  be  taken 
that  they  are  committed  in  time  to  an  insane,  or  epileptic  asylum. 


LECTURE    XXVII 

The  Psychoneuroses 

Gentlemen  :  When  in  the  following  lectures  I  describe  neurasthenia  and 
hysteria  in  particular  detail,  it  is.  in  the  first  instance,  from  thoroughly  practi- 
cal points  of  view.  There  can  be  no  doubt  that  the  importance  which  these 
two  most  wide-spread  nervous  diseases  have  in  the  work  of  the  physician, 
stands  in  striking  contradiction  to  the  neglect  under  which  thev  suffer  in  <n:n- 
eral  in  clinical  instruction.  The  disadvantage,  however,  which  insufficient  ac- 
quaintance with  these  disease  conditions  can  have  as  a  result  are  numerous 
and  serious.  Even  among  physicians,  the  view  of  the  laity,  which  in  the  symp- 
toms of  these  diseases  sees  evidence  of  an  affection  of  the  will,  an  expr  - 
of  effeminacy,  weakness  of  character,  contrary  disposition,  is  still  wide-spread, 
and  so  it  comes  about  that  such  patients,  for  whose  cure  the  first  requisite  is 
that  they  find  understanding,  lose  all  confidence  in  physicians  and  in  great 
numbers  fall  into  the  nets  of  quacks  and  charlatans  of  every  style  of  practice. 
Still  worse  is  it  indeed,  when,  as  unfortunately  so  often  happens,  in  the  making 
of  the  diagnosis  neurasthenia  or  hysteria,  wrong  is  done  and.  under  these  false 
colors,  organic  nervous  diseases  sail  <o  long  that  the  time  during  which  thera- 
peutic interference  is  possible  goes  by.  The  treatment  of  hysterical  and 
neurasthenic  conditions  is.  however,  when  correctly  carried  out.  on  the  a- 
a  grateful  task.  Here,  the  art  of  the  physician  may  accomplish  many  striking 
result-,  which  in  a  way  make  up  for  our  depressing  helplessness  when  opposed 
to  so  many  nervous  diseases.  Rut  the  difficulties  in  suitable  treatment  of  these 
diseases  also  are  not  to  be  underestimated.  This  preconceives  not  only  thor- 
ough knowledge  of  therapeutic  resources,  but  also  a  never-failing  interest  in 
the  disease  and  in  the  patient.  Such  prerequisites,  however,  are  absent  in 
only  too  many  physicians;  and  he  who  complains  about  his  "tiresome"  hysteri- 
cal and  neurasthenic  patients  cannot  reckon  upon  important  therapeutic  re- 
sults. I  will  attempt  to  show  you  that  neurasthenia  and  hysteria  are  anything 
but  tiresome.  Indeed  in  no  branch  of  medicine  i>  it  more  brought  to  our  con- 
sciousness that  our  specialty  is  not  only  a  technical  one.  but  to-day  still, 
not  deny  a  direct  descent  from  ^philosophy.  The  study  of  hysteria  and  neuras- 
thenia often  brings  US  into  contact  with  very  interesting  persons,  and  when 
tiny,  in  free  confession,  present  us  an  insight  into  their  psyche,  we  learn  to 
know  mechanisms  of  the  mental  processes  which  are  of  course  pathol 
but  in  general  bring  to  us  a  rich  harvest  in  knowledge  of  mankind  and  ps 
logical  understanding.  The  most  satisfactory  gain  remains,  however,  I  re- 
peat it  again,  to  therapeutic  effort  when  it  is  granted  to  us  to  remodel  un- 
fortunate individualities,  a  burden  to  themselves  and  others,  into  normally 
feeling  human  1m  ings. 

K)3 


404.  LECTURE    XXVII 


A.     Psychoneuroses  and  Neuropathic  Diathesis 

Neurasthenia  and  hysteria  are  still  much  classed  in  the  great  group  of 
"neuroses,"  as  which,  according  to  the  example  of  Cvllen,  the  Scotch  physician 
(1776),  are  brought  together  those  nervous  diseases  which  depend,  not  upon 
organic  alterations  of  the  nerve  cells  and  nerve  fibers,  but  usually  upon  func- 
tional abnormalities,  upon  an  unruly  activity  of  parts  of  the  nervous  system. 
Now,  however,  of  late  years  it  shows  itself  almost  from  day  to  day  more 
plainly  how  greatly  the  composition  of  this  group  does  violence  to  the  facts, 
and  how  entirely  heterogeneous  diseases  are  brought  in  such  manner  under 
a  common  classification.  The  group  of  the  neuroses  is  shrinking  more  and 
more,  and  this  is  indeed  natural,  since  their  definition  is  based  upon  a  negative 
peculiarity,  namely  the  want  of  an  anatomical  basis.  We  should  better  say, 
perhaps,  upon  our  lack  of  knowledge  of  such.  This  grouping  carries  in  itself 
the  stamp  of  the  provisory,  since  with  the  refinement  of  our  histological  tech- 
nique, and  the  progress  of  our  etiological  investigations,  alterations  up  to  this 
time  unsuspected  must  come  to  our  knowledge  and  also  toxic  and  infectious 
factors  will  be  disclosed.  As  some  decades  ago,  even  tabes  dorsalis  passed  for 
a  neurosis,  so  later  tetanus  and  hydrophobia  still  figured  under  this  designation, 
until  finally  their  microbic  nature  became  evident.  That  acromegaly,  Base- 
dow's disease,  tetany,  etc.,  have  as  a  basis,  anomalies  of  the  glandular  func- 
tions, is  a  recognition  of  recent  date,  which  must  result  in  their  exclusion  from 
the  nosological  group  "neuroses."  As  far  as  epilepsy  is  concerned,  I  have  laid 
down  to  you  in  the  last  lecture,  that  to-day  a  doubt  as  to  its  nature  as  an 
organic  affection  of  the  brain-cortex  is  no  longer  possible,  and  that  we  must 
assume,  where  no  histological  changes  could  be  recognized,  that  it  was  the 
fault  of  the  insufficiency  of  technique;  the  same  conclusion  is  permitted  ii} 
chorea. 

That  on  the  other  hand  in  neurasthenia  and  hysteria,  as  a  matter  of  fact, 
no  anatomical  alteration  has  ever  been  found,  would  not  suffice  in  my  opinion 
to  prove  them  the  definite  survivors  from  the  family  of  the  neuroses;  this  still 
could  depend  upon  the  weakness  of  our  microscopes  and  the  imperfection  of  our 
staining  methods.  But  there  are  also  positive  signs  common  to  both  affections. 
To  be  emphasized  above  every  thing  are  the  transitoriness  and  changeability, 
the  proteus-like  manifoldness  in  the  succession  and  in  the  combination  of  the 
symptoms,  which  can  make  themselves  apparent  not  only  from  case  to  case,  but 
also  in  one  and  the  same  patient  from  day  to  day,  often  from  hour  to  hour, 
while  in  other  nervous  diseases,  the  clinical  pictures  can  repeat  themselves  from 
case  to  case  with  a  stereotyped  similarity ;  for  the  troubles  of  neurasthenics  and 
hysterics,  if  a  paradox  is  permitted  to  me,  the  sole  rule  which  prevails  is  that 
of  irregularity. 

Still  more  important,  however,  is  a  peculiarity  taking  these  disease  con- 
ditions out  of  the  limits  of  other  nervous  diseases,  I  mean  the  greatly  pre- 
ponderant role  which  in  their  origin,  in  the  method  of  appearance  of  their 
symptoms,  and  on  account  of  this  also  in  the  curative  procedures  adapted  to 
combating  them,  is  played  by  psychic  injuries,  psychic  phenomena,  and  psychic 


THE    PSYCHONEUROSES  405 

influences.  On  this  account  we  accept  the  denomination  "psychoneuro'ses" 
which  Paul  Dubois  has  introduced  for  these  disease  conditions.  This  word, 
indeed,  expresses  in  appropriate  manner  that  the  whole  multiplicity  of  "'ner- 
vous symptoms,"  even  where  they  manifest  themselves  in  the  bodily  sphere,  is 
most  closely  connected  with  mental  factors ;  in  their  occurrence,  emotions, 
hypochondriac  fears,  exaggerated  self-observation,  play  an  important  role, 
as  do  also  the  suggestive  effects  of  surroundings  or  the  abnormal  products  of 
a  phantasy  directed  into  false  paths. 

Welcome  to  us  further  is  the  term  psychoneurosis  as  a  common  designation 
for  the  two  disease  conditions  in  question.  In  these  latter,  the  sentence  "natura 
non  facit  saltum"  (nature  does  not  take  a  leap),  is  especially  applicable.  The 
cases  in  which  we  are  in  doubt  as  to  whether  we  shall  diagnose  hysteria  or 
neurasthenia,  in  which  we  must  assist  ourselves  with  the  designation  "hystero- 
neurasthenia"  and  where  transition  and  intermediate  forms  of  these  diseases  are 
present,  are  excessively  frequent.  The  boundaries  in  any  case  are  not  to  be 
drawn  sharply,  and  if  we  do  draw  them,  this  is  chiefly  upon  didactic  grounds, 
since  without  boundary  marks  to  some  extent  definite,  we  could  not  locate 
ourselves  in  the  confusion  of  symptomatology. 

In  tiresome  monotony  there  meets  us,  over  and  over  again,  the  common- 
place, that  the  psychoneuroses  (or  as  the  popular  equivalent  has  it,  "nervos- 
ity") are  diseases  of  modern  civilization.  This  is  applicable  only  with  limita- 
tions, since  hysteria,  and  indeed  in  its  most  severe  and  striking  forms,  is  as  old 
as  humanity.  This  must  be  plainly  apparent  to  every  one  who  knows  how  to 
estimate  in  their  proper  nature  the  hysterical  epidemics  of  ancient  times  and 
of  the  Middle  Ages,  and  Hippocrates,  even,  has  furnished  good  descriptions  of 
hysterical  symptoms,  which  he  also  recognized  as  such.  It  is  somewhat  differ- 
ent, indeed,  with  neurasthenia;  though  there  are  neurologists  who  speak  of  the 
'"discovery"  of  this  disease  by  the  American  George  Beard  in  the  year  1887. 
Of  course  this  author  did  not  discover  neurasthenia,  hut  studied  and  described 
it  in  masterly  fashion,  recognized  it  in  its  nature,  and  furnished  it  with  a 
fortunately  chosen  name.  When,  however,  we  look  over  the  medical  literature 
of  preceding  decades,  we  find  under  other  designations  (such  as  "nervosism," 
"nervous  erythism,"  "general  hyperesthesia,"  "cerebro-cardial  neuropathy," 
"spinal  irritation")  disease  pictures  which  present  typical  neurasthenic  symp- 
tom-complexes; particularly,  however,  is  it  the  diagnosis  hypochondria  with 
which  since  ancient  times,  neurasthenia  lias  been  saddled.  With  the  old  authors 
hypochondria  played  a  preponderant  role;  to-day  we  make  this  diagnosis  only 
in  rare  cases  in  which  there  is  a  mental  disease  proper,  with  thoroughly  sys- 
tematized delusional  conceptions  built  up  upon  fears  of  disease.  The  picture, 
however,  which  Moliere  in  his  "Malade  Imaginaire"  draws  of  a  presumable 
hypochondriac,  is  that  of  nothing  else  hut  an  excellently  observed  and.  in  spite 

of    caricature,    unmistakable    neurasthenic.       The    disease    which    in    the    seven 
teenth    century   under    the    popular   name    "Vapors,"    scourged    the    Court    ami 
noble  circles  of  France,  was  scarcely  anything  else;  in  the  letters  of  Mme.  de 
SSvignS  and  of  the  Abbi  de  Brosse  then   are  proofs  of  this.     It  would  lead  me 

too  far  afield  to  enter  into  the  documents  bearing  upon  the  history  id'  neuras- 
thenia furnished  by  the  ancient  writers;  for  example,  the  Roman  satirists,  but 


406  LECTURE    XXVII 

I  cannot  fail  to  cite  a  clinical  history  taken  from  the  fifth  Hippocratic  book, 
"On  Epidemics."  This  was  of  a  patient  "Who  suffered  from  bodily  relaxa- 
tion. He  could  not  pass  by  any  pit,  neither  could  he  go  on  a  bridge,  nor  yet 
was  he  in  a  condition  to  cross  over  even  the  slightest  trench,  although  he  could 
walk  in  the  trench  itself.  Such  things  happened  to  him  for  a  very  long  time." 
You  see  here  the  unmistakable  sketch  of  obsessive  ideas  which  are  so  frequent 
in  severe  neurasthenia.  The  description  of  "Gephyrophobia,"  as  we  term  it 
to-day,  is  indeed,  classical. 

Taken  as  individual  diseases  the  psychoneuroses  need  not  be  designated  as 
the  regrettable  privilege  of  our  present  epoch  of  culture.  Now  at  present, 
however,  that  is  for  about  the  last  three  decades,  we  have  not  alone  to  deal 
with  single  cases  or  with  epidemics  bounded  by  time  and  place,  but  with  a 
definite  nervosity  of  peoples  and  races  which  although  not  dangerous  to  life, 
is  nevertheless  of  great  importance,  since  it  injures  intensely  the  capacity  for 
work  and  productivity  of  extended  strata,  of  all  classes  of  society.  Into  the 
causes  of  this  threatening  increase  of  the  psychoneuroses  I  will  not  enter  at 
the  start.  We  will  reserve  the  pathogenetic  considerations  for  the  next  lecture. 
In  order  to  give  you,  however,  a  comparative  numerical  conception  as  to  the 
extent  of  the  psychoneuroses,  I  would  refer  to  the  investigations  of  Cramer 
among  the  Gottingen  student  body.  By  comparison  of  the  whole  number  of 
students  with  the  number  of  those  who  sought  professional  advice  on  account 
of  nervosity,  he  found  the  figures  of  30  to  40  per  cent.,  and  thinks  that  these 
figures  are  certainly  not  too  high,  since  the  students  are,  with  few  exceptions, 
individuals  who  have  as  yet  been  little  shaken  up  by  the  storms  and  experiences 
of  life.  On  the  other  hand,  however,  I  might  point  out  that  among  students, 
other  nerve-injuring  factors  come  into  action  in  a  relatively  high  percentage 
of  cases ;  fear  as  to  examinations,  venereal  diseases,  excesses  in  drinking,  etc. 
In  any  case  the  comparison  drawn  by  Cramer,  with  the  frequency  of  psychoses, 
is  interesting;  among  1,000  people  there  are  2  insane  persons  needing  asylum 
care,  and  2  who  can  more  or  less  get  along  in  the  world. 

Shall  we  class  the  psychoneuroses  with  the  congenital  or  with  the  acquired 
diseases?  This  question  I  can  answer  you  neither  in  the  affirmative  nor  in  the 
negative.  It  is  true  that  on  the  one  hand  external  injurious  factors  can  pro- 
duce neurasthenia  and  hysteria;  on  the  other,  however,  that  for  many  cases  the 
manifestation  of  an  inferiority  present  in  the  organism  from  the  start  is  re- 
sponsible. Between  the  congenital,  endogenic,  and  the  acquired  exogenic  fac- 
tors, there  is  only  a  relation  of  reciprocity.  External  injurious  factors  of 
considerable  intensity  can  make  even  an  individual  who  possesses  from  the  start 
a  robust  nervous  system  neurasthenic  or  hysterical.  In  others,  however,  witli 
the  best  will  and  through  the  most  exact  anamnesis,  we  cannot  elicit  any  ade- 
quate external  injurious  factors;  here  it  is  to  be  assumed  (and  often  to  be 
recognized)  that  such  individuals  have  brought  into  the  world  a  nervous  sys- 
tem having  abnormal  lack  of  resistance,  so  that  they  are  unable  to  withstand 
the  demands  of  life,  even  on  the  school-bench,  or  still  earlier,  and  fall  ill  under 
conditions  which  to  one  healthy  nervously,  "ab  ovo,"  would  be  entirely  harmless 
and  irrelevant. 

We  come  here,  also,  as  in  so  many  branches  of  pathology,  upon  the  con- 


THE    PSYCHONEUROSES  407 

ception  of  "predisposition."  In  this  special  case  this  is  denominated  "neuro- 
pathic diathesis."  This  conception  has  been  much  ridiculed,  since  it  contains 
in  itself  much  that  is  vague  and  unsatisfactory,  but  even  the  most  exact  branch 
of  medicine,  bacteriology,  has  had  to  recognize  that  it  cannot  get  along  with- 
out the  conception  of  predisposition.  Only  in  this  way  can  we  understand  all 
the  remarkable  leaps  which  an  epidemic  makes,  in  that  it  affects  one  individual, 
spares  another,  although  it  is  known  that  this  last  one  has  been  exposed  in  a 
high  degree  to  the  danger  of  infection. 

Close  relationships  join  the  question  of  neuropathic  diathesis  with  that 
of  "hereditary  predisposition."  One  can  well  say  to-day  that  the  importance 
of  this  last  is  greatly  overestimated  in  the  occurrence  of  the  psychoneuroses. 
It  has  been  implicitly  assumed  that  the  healthy  person  must  be  free  from 
"hereditary  predisposition."  This  is,  however,  not  so.  J.  Roller  has  found 
that  among  the  relations  of  370  healthy  persons,  different  neuroses  and  psy- 
choses were  to  be  found  218  times,  which  corresponds  to  a  percentage  of  59. 
These  figures,  high  beyond  expectation,  make  for  the  critical  observer  the  as- 
sumption of  a  sure  causal  nexus  between  the  psychoneurotic  disease  of  an 
individual  and  the  neuro-  and  psycho-pathies  occurring  in  his  family,  a  matter 
of  opinion  which  must  in  every  individual  case  be  particularly  weighed  and 
considered.  Also  it  must  continually  be  emphasized  that  from  an  entirely 
healthy  stem  neuropathic-ally  inclined  offspring  may  be  produced.  Here,  now, 
in  relatively  numerous  cases  the  "blastophthoric"  injurious  factors  of  which 
we  spoke  in  Lecture  VIII  are  to  be  found  among  the  ascendants.  In  severe 
cases  of  neuropathic  diathesis  somatic  signs  of  congenital  inferiority  appear  to 
us  in  the  form  of  the  so-called  stigmata  of  degeneration  to  whose  importance 
Lombroso,  in  his  studies  on  "The  Congenital  Criminal,"  lias  referred.  We 
consider  as  such  stigmata:  Marked  asymmetry  of  the  skull  not  to  be  explained 
by  exogenic  factors  (for  example  injury  at  birth),  anomalies  of  the  teeth, 
greal  prominence  of  the  upper  jaw  or  of  the  lower  jaw  (prognathism,  caput 
progeneum,  Gothic  palate,  cleft  palate,  harelip),  anomalies  of  the  external 
ear  (jughandle  ear,  adherent  lobe,  pointed  ear),  hypospadias,  genital  infantil- 
ism, syndactylism,  web  fingers,  gampsodactylism  (inability  to  extend  the  little 
finger),  etc. 

B.      Neurasthenia 

Since  we  have  touched  upon  the  most  important  points  which  should  pre- 
pare the  way  for  the  understanding  of  psychoneurotic  manifestations  in  gen- 
eral, we  will  pass  over  to  the  description  of  those  disease  pictures  which  we 
bring  together  as  neurasthenic;  those  of  hysteria  we  will  reserve  until  later, 
since  tiny  are  mainly  more  complicated  and  more  difficult  to  understand.  Mid 
for  the  present  in  the  consideration  of  the  neuropathic  phenomena  we  will  put 
aside  all  theoretical  and  all  pathogenetical  considerations  and  make  our  de- 
scription purely  clinical. 


408  LECTURE    XXVII 

Symptomatology 

The  disease  manifestations  of  neurasthenia  separate  into  objective  and  sub- 
jective, of  which  the  last  play  a  specially  important  role.  On  account  of 
this,  the  result  of  the  anamnesis  is  just  as  instructive  as  that  of  the  status, 
and  we  have  to  devote  particular  attention  to  hearing  what  the  patient  has  to 
say.  In  this,  however,  a  certain  routine  is  an  absolute  requirement.  It  indeed 
is  one  of  the  peculiarities  of  the  neurasthenic  to  enter  with  the  utmost  detail 
into  each  single  symptom  of  his  disease,  and  he  usually  makes  no  difference, 
or  no  sufficient  difference,  between  the  important  and  the  unimportant.  Noth- 
ing is  more  unwise  than  to  cut  him  off  shortly,  when  his  conversation  is  super- 
fluously long.  The  neurasthenic  takes  this  very  ill,  and  a  physician  who  is  im- 
patient, or  more  correctly,  who  gives  evidence  of  his  impatience,  will  rapidly 
lose  confidence  and  authority  with  such  patients.  One  must  hence  learn  to  cur- 
tail the  prolixity  of  his  description  of  his  disease,  by  properly  interpolated 
questions,  turning  the  narrator  away  from  the  unimportant  and  guiding  him 
to  the  symptoms  diagnostically  important.  A  considerable  fraction  of  neuras- 
thenics frequently  aid  us  in  taking  the  anamnesis  and  compiling  the  clinical 
history,  in  that  they  hand  us  written  sketches  of  their  disease  (in  which  no 
symptom  is  unconsidered  or  forgotten)  ;  "l'homme  aux  petits  papiers"  (the 
man  with  the  little  papers)  Charcot  hence  has  called  the  neurasthenic. 

Let  me  place  before  you  two  examples  of  such  auto-nosographies  with  the 
remark  that  we  cannot  expect  always  such  short  and  expressive  descriptions: 

1.  '"Symptoms. — With  ever  so  slow  increase  of  severe  pains  in  the  chest 
and  in  the  back  from  the  neck  to  the  anus.  Burning  over  the  whole  body,  par- 
ticularly severe  in  the  legs,  a  biting  feeling  over  the  whole  head.  Three  and 
one-half  to  four  hours  rest  at  night,  then  sudden  awakening,  trembling  over 
the  whole  body  and  no  more  sleep ;  sweating  at  night,  by  day  very  frequent 
nervous  tremor,  anxiety,  uncertainty  in  walking.  Attacks  of  vertigo  very 
frequent." 

2.  "Remarks  for  the  Doctor. — Often  headaches  and  sleepless  nights. 
Short  dizziness,  a  feeling  as  if  the  head  was  drawn  backward,  pain  when  there 
is  noise,  starting  at  every  noise,  for  example,  the  fall  of  a  tablespoon.  One 
morning  I  felt  a  stiffness  on  the  left  side,  felt  myself  strange  in  my  own  house, 
the  people  and  everything  appeared  to  me  different.  Little  by  little  the  old 
impressions  returned  to  me,  only  memory  remained  poor.  Visually  the  stiff- 
ness on  the  left  side  repeats  itself,  indeed  it  is  noticeable  also  on  the  right. 
Sometimes  stabbing  headache  and  on  strenuous  work,  sense  of  nausea.  An  ex- 
ceedingly painful  uncertainty  pursues  me  the  whole  day,  then  I  question  myself, 
must  I  do  that,  is  it  I  or  only  a  dream,  and  the  interpretation  is  now  obscure- 
in  my  head.     I  cannot  any  longer  collect  my  thoughts." 

These  two  typical  personal  observations  respectively  of  a  patient  with 
principally  somatic  symptoms  and  of  one  with  predominantly  psychic  symp- 
toms ("psychasthenia")  show  how  extremely  manifold  the  symptoms  of  the 
psychoneuroses  now  occupying  us  can  be.  It  is  hence  well  for  us  to  proceed 
quite  systematically  to  bring  the  greatest  possible  order  out  of  this  confusion 
and  to  subject  the  chief  phenomena  of  neurasthenia  to  a  separate  considera- 


THE    PSYCHOXEUROSES  409 

tion.  We  will  suppose  a  fully  developed  case  of  acquired  neurasthenia,  that 
is,  in  which  the  special  congenital  degenerative  characteristics  do  not  occupy 
the  foreground.  The  degenerative  forms  will  later  be  discussed  as  well  as  a 
few  semiologically  characteristic  varieties,  such  as  the  so-called  '"sexual  neuras- 
thenia." 

Since  in  the  course  of  talcing  the  anamnesis  we  become  acquainted  with  the 
subjective  complaints  while  only  examination  into  the  present  condition  brings 
the  objective  symptoms  before  us,  it  is  justifiable  to  begin  our  description  with 
the  first  group  of  disease  manifestations. 

1.     PSYCHIC    ANOMALIES 

a.      State  of  Feeling 

The  basal  symptom  in  the  state  of  feeling  of  the  neurasthenic  is  what  George 
'Beard  has  characterized  as  irritable  weakness,  an  abnormal  irritability  and 
exhaustibility.  The  patient  is  irritable;  trifles  ("the  flies  on  the  wall")  disturb 
him.  To  disagreeable  occurrences,  or  to  actual  mental  pain  he  reacts  in  im- 
moderate manner,  after  the  emotional  discharge  to  sink  back  into  deep  apathy. 
Even  agreeable  happenings,  pleasurable  stimuli,  produce  excessive  reaction, 
and  then  weakness  and  exhaustion  often  with  psychical  depression.  To  "sky- 
high  exaltation"  succeeds  "'mortal  depression."  Still  the  basal  state  of  feeling 
upon  which  such  episodes  arise  is  usually  a  comparatively  depressed  one.  rather 
morose  than  sad.  This  ill  humor  is  in  the  main  to  be  considered  as  a  second- 
ary symptom  corresponding  to  the  uncomfortable  general  condition;  and  re- 
flections about  the  diseased  ego  play  in  this  a  considerable  role.  In  any  case, 
it  is  a  quite  different  symptom  from  the  depression  of  the  melancholiac,  since 
this  last  in  no  way  arises  from  reflection,  but  without  any  thought  as  to  its 
cause,  it  impresses  itself  upon  the  patient  with  overpowering  might.  Anxiety 
mainly  of  a  hypochondriacal,  sometimes  also  of  a  superstitious  coloring,  as 
well  as  defect  of  energy  and  inability  to  form  conclusions,  are  also  quite  char- 
acteristic. 

b.     Intellectual  Capabilities 

In  tlir  intellectual  sphere  on  the  other  hand,  no  important  disturbances 
arc  noticeable  except  for  the  abnormally  rapidly  occurring  and  tin-  abnormally 
disagreeably  perceived  fatigability,  which  gives  to  the  mental  work  of  the 
highly    endowed    and    talented    persons,    who    form    no    small    proportion    of    pa 

tiente  with  acquired  neurasthenia,  an  aphoristic  ami  broken-off  character.  In 
any  event  the  patients  often'  complain  of  "reduction  of  their  mental  force," 
that  flu'  ability  to  receive  and  combine  impressions  and  the  memory  have  suf 
fered.  A  more  exact  investigation,  however,  makes  it  plain  to  us  that  these 
functions  are  in  fad  uninfluenced,  and  that  only  the  continued  occupation  of 

the  patient  with  the  observation  and  explanation  of  his  troubles  prevents  him 
from  turning  his  attention  sufficiently  to  things  of  the  surrounding  world. 
This  "'narrowing  of  the-  field  of  consciousness,"  brings  it  about  that  a  neuras- 
thenic must  read  the  same  sentence  several  times  before  he  understands  it,  that 


410  LECTURE    XXVII 

nanus,  words,  dates,  easily  escape  him  (since  he  has  imperfectly  noted  them), 
that  he  is  unable  to  concentrate  his  thoughts  strictly  upon  an  intellectual  task. 

2.    HEADACHES 

More  than  half  of  all  neurasthenic  patients  complain  of  headache.  If  we 
require  them,  however,  which  we  should  not  neglect  in  any  case,  to  give  an 
accurate  description  of  this  trouble,  we  will  usually  find  that  there  are  not 
actual  pains,  but  only  abnormal,  more  or  less  unpleasant  sensations.  Mainly 
we  have  to  do  with  what  Charcot  called  "le  casque  neurasthenique"  (neuras- 
thenic helmet),  a  feeling  of  a  weight  pressing  upon  the  whole  skull.  Frequently 
the  pressure  is  located  only  in  the  frontal  region,  more  rarely  at  the  back  of 
the  neck.  Other  patients  complain  of  a  feeling  that  the  brain  is  threatening 
to  burst  the  skull,  or,  on  the  contrary,  that  it  is  too  small,  that  the  head  is 
empty.  Often  it  is  only  a  "dullness  in  the  head."  Of  true  severe  headaches 
only  such  neurasthenics  who  live  in  terror  of  suffering  from  a  severe  organic 
brain  disease,  as  brain  tumor  or  brain  syphilis,  complain  usually.  Neverthe- 
less, such  head  troubles  increased  in  consequence  of  intense  fixation  of  the  at- 
tention and  autosuggestive  factors  to  considerable  intensity,  are  to  be  recog- 
nized as  to  their  neurasthenic  nature  before  exact  investigation,  by  the  fact 
that  upon  the  mental  abstraction  of  the  patient  they  disappear. 

3.     SLEEPLESSNESS 

This,  also,  constitutes  an  exceedingly  frequent  complaint  of  neurasthenics; 
still,  their  expression  "sleeplessness"  is  never  to  be  taken  literally,  even  al- 
though the  patients  expressly  insist  that  they  cannot  sleep  at  all,  "hear  every 
quarter  hour  strike."  In  such  cases  one  has  to  do,  as  a  rule,  only  with  an 
insufficient  depth  of  sleep,  with  a  condition  of  half  sleep,  which  we,  with  the 
Hungarian  author  Lechner,  who  has  studied  the  disturbances  of  sleep  particu- 
larly thoroughly,  will  designate  "Dysnystaxis."  Other  patients  suffer  from 
difficulty  in  falling  asleep  ("Dyskoimesis"),  or  from  too  early  awakening 
("Dysphylaxia")  although  sleep  is  deep  enough.  Distressing  dreams,  night- 
mare, are  not  rare.  The  patients  very  often,  indeed,  complain  of  a  sudden 
starting  which  frightens  them  just  as  they  are  going  to  sleep.  Even  when 
the  duration  and  the  depth  of  sleep  apparently  present  no  particular  anomalies, 
the  refreshing  action  of  sleep  is  absent  and  we  hear  then  that  the  patients 
"arise  more  tired  than  they  were  when  they  went  to  bed."  Especially  trying 
is  a  drowsiness  during  the  day  which  is  in  great  contrast  with  the  abnor- 
mal wakefulness  at  night.  "Night  is  the  enemy  of  the  nervous,"  correctly 
says  Hermann  Oppenheim,  who  has  subjected  the  genesis  of  the  neurasthenic 
disturbances  of  sleep  to  a  keen  analysis.  With  the  abandonment  of  activity 
and  all  external  interests,  the  attention  of  the  neurasthenic  may  be  turned  in  a 
higher  degree  to  his  bodily  processes,  so  that  there  occurs  a  hyperesthesia 
toward  sensations  which  the  healthy  person  does  not  perceive  at  all,  for  in- 
stance, his  own  heart-beat,  the  peristalsis  of  his  intestines.  These  continued 
sense  perceptions  naturally  oppose  going  to  sleep,  for  which   the  elimination 


THE    PSYCHONEUROSES  411 

as  far  as  possible  of  the  irritants  reaching  the  sensorium  furnishes  the  best 
conditions.  Through  a  specially  exaggerated  "self-perception"  every  organic 
feeling  can  take  on  an  actually  painful  coloring,  on  account  of  which  we  speak 
of  "  hypnalgias"  and  "nyctalgias."  The  healthy  person  also  for  all  that,  can 
provoke  such  phenomena  in  himself  by  way  of  experiment.  Attempt  during 
the  stillness  and  darkness  of  the  night  when  your  attention  cannot  be  dis- 
tracted by  external  perceptions  to  fix  this  in  the  greatest  concentration  pos- 
sible upon  a  definite  part  of  your  body,  for  example,  upon  one  heel;  you  will 
in  a  few  minutes  perceive  an  unpleasant  sensation  in  this  region.  The  contact 
with  the  sheet  will  be  first  found  noticed  as  an  unpleasant  tickling,  then  as 
itching  and  burning,  and  in  case  you  do  not  allow  your  attention  to  be  dis- 
tracted in  some  other  direction,  finally  as  actual  pain.  Upon  the  basis  of  this 
personal  experiment  which  can  be  repeated  without  difficulty,  I  cannot  agree 
with  Oppenheim  when  he  writes  that  the  interpretation  of  hypnalgias  and 
nyctalgias  gives  particular  difficulty. 

4.     IRRITATIVE   SYMPTOMS  ON  THE   PART  OF  THE 
SENSE    ORGANS 

Exaggerated  feeling  of  self  furnishes  us  the  key  to  the  understanding  of 
a  number  of  subjective  ear  and  eye  symptoms  which  occur  in  a  large  number 
of  neurasthenics  and  in  which  we  are  justified  in  assuming  a  morbid  irritability 
of  the  sense  nerves  in  question,  a  reduction  of  their  threshold  of  irritability. 
The  following  irritative  symptoms  are  to  be  observed:  Hyperesthesia  to  light, 
blinded  feeling,  spots  before  the  eyes,  hyperesthesia  for  noise,  so  that  such 
patients,  for  example,  perceive  the  whistle  of  a  locomotive  as  actual  pain  and 
anxiously  avoid  one.  Further,  tinnitus  auriurn,  sometimes  rhythmic,  synchro- 
nous with  the  pulse,  and  in  this  case  dependent  upon  the  perception  of  the  noise 
of  the  circulation  within  the  skull,  to  which  the  cochlear  nerve  with  normal 
threshold  of  irritability  would  not  react.  The  entoptic  perception  of  neuras- 
thenics, denominated  "musci  volitantes,"  has  nothing  to  do  with  similar  phe- 
nomena in  opacities  of  the  vitreous  body  (indeed,  ophthalmoscopic  examination 
Shows  hen-  tli.it  all  the  media  of  the  eye  are  clear),  and  is  probably  produced 
by  the  circulation  in  the  retina  or  by  the  corpuscular  elements  of  the  tear 
secretion.  Acuteness  of  vision  and  hearing  remains  uninfluenced  in  all  the 
phenomena  mentioned.  The  single  defect  symptom  which  neurasthenia  may  pro- 
duce in  the  visual  apparatus  is  usually  to  be  referred  to  the  abnormal  fatig- 
ability of  the  internal  recti  and  of  the  ciliary  muscle;  these  are  the  SO-Called 
"asthenopic"  troubles  which  make  themselves  apparent  after  a  more  or  less 
.short    time,   upon   fixation   and   accommodation    for   near   objects. 

5.     VERTIGO 

Only  in  quite  rare  cases  do  Inn  attacks  of  vertigo  with  disturbances  of 
equilibrium   occur   (for  example   having   a    rotatory    vertiginous   character), 

which  can  be  considered  as  an  irritative  symptom  on  Hie  part  of  a  pathologi- 
cally   irritable    vestibular    apparatus,    wliieli    answers    in    this    manner    to    Midden 


412  LECTURE    XXVII 

variations  in  the  blood  distribution  in  the  internal  car.  When,  in  spite  of  this, 
our  neurasthenic  patients  complain  of  vertigo  with  excessive  frequency,  this 
comes  from  the  fact  that  under  this  term,  the  public  understands  very  different 
things.  A  momentary  fit  of  weakness,  the  feeling  that  it  is  suddenly  black 
before  the  eyes,  as  if  the  legs  were  sinking  into  the  floor,  or  as  if  they  were 
taken  away  from  the  body,  all  these  abnormal  sensations  generally  come  under 
this  designation.  If  a  neurasthenic  has  once  experienced  a  subjective  difficulty 
of  this  sort  the  fear  of  "vertigo"  usually  makes  itself  felt  in  very  marked  form 
and  the  reoccurrence  of  such  symptoms  naturally  increases  considerably  this 
fear,  a  painful,  vicious  circle.  Fortunately  in  very  few  cases  does  an  actual 
"jaermanent  vertigo"  appear. 

6.     PARESTHESIAS 

Abnormal  sensations  occur  in  neurasthenics  not  only  in  the  regions  of  the 
skull  and  the  nerves  of  special  sense,  but  also  in  those  of  the  skin,  the  mucous 
membranes,  muscles,  bones,  tendons  and  joints,  in  varied  and  manifold  forms. 
If  they  are  also  of  unpleasant  character  and  indeed  actually  painful,  so  is  there 
here  also,  as  we  emphasized  under  the  "Headache"  and  "Sleeplessness"  of 
neurasthenics,  very  frequently  an  unconscious  tendency  to  exaggeration.  Not 
at  all  infrequently  such  patients  complain  of  their  "terrible"  pains  not  ceasing 
a  moment,  but  their  healthy  appearance,  their  expression,  their  abstractibilit y, 
the  fact  that  they  can  exercise  their  calling  undisturbed,  etc.,  form  such  a  con- 
trast to  the  patients  who  suffer  from  the  lancinating  pains  of  tabes  or  trige- 
minal neuralgia,  that  we  are  in  a  position  without  difficulty  to  reduce  their 
description  to  its  proper  proportion.  The  most  frequent  seat  of  such  pain- 
ful paresthesias  is  the  back;  we  speak  then  of  spinal  irritation,  of  rachialgia. 
But  also  in  the  muscles  of  the  limbs  are  located  various  sensations  which 
probably  correspond  to  those  which  the  normal  person  perceives  after  intense 
exertion  as  the  well-known  "fatigue  pain."  In  rare  cases  "dysbasia  neuras- 
thenica  intermittens,"  which  should  not  be  confounded  with  the  angiogenic, 
forms  of  intermittent  limping,  can  so  occur  (see  above,  pages  225  and  374). 
Here,  probably,  the  two  components  of  "irritative  weakness"  (morbid  increase 
of  motor  fatigability  on  the  one  hand  and  of  sensory  irritability  on  the  other) 
play  an  important  role.  As  paresthesia  of  the  bones  is  to  be  interpreted  the 
so-called  "anxietas  tibiarum,"  a  dull,  difficultly  definable  feeling  in  the  bones  of 
the  leg — whose  parallel  occurring  in  healthy  people  is  the  well-known  sensation 
designated  by  the  expression,  "the  fright  has  got  into  my  legs" — which  can  be 
experienced  continuously  by  the  neurasthenic.  Neurasthenic  arthralgias  have 
led  to  confusion  with  rheumatic  joint  diseases.  In  contradistinction  to  these 
last,  however,  they  increase  in  intensity  during  rest,  diminish  on  movement,  a 
criterion  which  applies  to  many  neurasthenic  pains,  as  Kollorits  has  expressly 
emphasized.  Pruritus,  itching  in  the  skin  and  mucous  membranes  forms,  finally, 
a  frequent  complaint  of  neurasthenics.  It  is  almost  never  general,  as  are  often 
the  parasitic,  the  senile,  and  the  forms  occurring  in  diabetics  and  arteriosclero- 
tics;  it  is  rather,  almost  always,  localized  upon  definite  areas.  Itching  of  the 
pharyngeal  mucous  membrane  leads  to  dry,  nervous  "irritative  cough,"  pruri- 


THE    PSYCHONEUROSES  413 

tus  of  the  urethral  or  rectal  mucous  membrane  to  distressing  tenesmus  of  these 
excretory  passages;  pruritus  of  the  genitals  may  be  the  starting-point  for 
masturbatory  practices.  It  is  self-evident  that  the  diagnosis  of  nervous  pruri- 
tus should  only  be  made,  when  an  exact  investigation  can  exclude  the  other 
disease  conditions  above  mentioned. 

When  we  turn  our  attention  to  the  subjective  symptoms  of  neurasthenia. 
we  are  first  expressly  reminded  of  the  polymorphism  of  the  neurasthenic  disease 
picture.  This  brings  it  about,  that  we  need  not  expect  in  every  neurasthenic 
to  find  represented  each  of  the  four  groups  of  symptoms  into  which,  upon 
didactic  grounds,  it  is  well  to  arrange  these  anomalies.  Still,  almost  never 
will  a  neurasthenic  fail  to  show  any  objective  symptoms  at  all,  so  that  their 
diagnostic  importance  is  not  to  be  underestimated. 


7.     INCREASED    IRRITABILITY    OF    THE    NEUROMUSCULAR 
APPARATUS 

This  anomaly  expresses  itself  particularly  in  a  general  increase  of  the 
bone  and  tendon  reflexes,  which  indeed  in  general  remains  below  that  which 
we  find  in  organic  affections,  for  example,  in  the  spastic  symptom-complex, 
but  still  is  occasionally  so  intense  that  a  doubt  may  arise  in  this  direction. 
In  such  cases,  the  fact  that  functional  increase  of  reflexes  is  never  accom- 
panied by  Babinski's,  Oppenheim's,  or  the  Mendel-Bechterew  phenomenon,  asso- 
ciated movements,  etc.,  is  of  great  importance.  Further,  permanent  hyper- 
tonia of  the  muscles  is  absent.  The  so-called  ''functional"  or  "pseudo"  ankle 
clonus  could  at  most  give  occasion  for  confusion.  Still,  there  are  never  here 
the  thoroughly  rhythmic,  even  contractions  which  can  lie  provoked  as  long 
as  desired,  which  characterize  true  organic  ankle  clonus,  but  irregular  move- 
ments of  varying  amplitude,  which,  besides,  are  soon  exhausted.  This  is 
shown  most  plainly  in  graphic  reproductions  of  true  and  false  clonus  upon 
Up-  rotating  drum  ( clonograph ) ,  still,  an  examiner  of  any  experience  can  al- 
ways dispense  with  this  complicated  method  of  investigation.  A  reduction 
of  the  tendon  reflexes  can  some!  inns  he  found  in  such  neurasthenics  who,  in 
consequence  of  marked  nervous  dyspepsia,  are  in  a  condition  of  considerable 
muscular  emaciation. 

The  absence  of  a  reflex  phenomenon  normally  constant,  particularly  also 
of  the  patellar  reflex,  in  a  neurasthenic,  indicates  without  exception  a  com- 
plicating organic  anomaly.  Also  the  skin  reflexes,  namely  the  plantar  reflex, 
are  in  neurasthenics  sometimes  exaggeratedly  lively. 

Frequently  an  increased  mechanical  muscle  irritability  is  to  hi'  found,  so 
thai  even  slight  blows  with  the  percussion  hammer  upon  the  extensor  side 
of  the  forearm  set  up  a  powerful  motor  ell'ect  at  the  wrist  and  in  the  indi- 
vidual fingers.  Also  in  the  quadriceps  and  in  the  pectoralis  major  is  this 
mechanical  hyper-excitability  of  tin-  muscles  often  very  clearly  to  In-  recog- 
nized. The  "idiomuscular  contraction"  is  sometimes  very  plainly  visible.  On 
the  other  hand,  mechanical  o\  erirril  aliilit  y  of  the  nerve  trunks  is  iniieh  rarer. 
It  is  most   frequently   demonstrable  upon   the  ulnar   nerve   In  hind   the  elbow, 


•114.  LECTURE    XXVII 

in  that  by  rolling  this  nerve  under  the  palpating  finger  a  definite  contraction 
of  all  the  muscles  supplied  by  it  can  be  produced. 

8.     TREMOR   AND    SIMILAR    PHENOMENA 

In  the  great  majority  of  neurasthenics,  when  we  request  the  patient  to 
close  the  eyes  firmly  we  can  notice  a  marked  tremor  in  the  orbicularis  palpe- 
brarum. The  slightest  degrees  of  this  phenomenon  are,  as  you  should  note, 
presented  sometimes  by  an  entirely  healthy  person,  for  instance,  in  a  condition 
of  fatigue,  after  long  reading;  in  the  neurasthenic,  however,  this  may  increase 
to  a  blinking,  which  is  increased  upon  attempt  to  keep  the  eyelids  as  still  as 
possible.  Less  frequent  are  tremor  of  the  outstretched  tongue,  and  that  of  the 
fingers  which  is  usually  very  fine  and  vibrating.  Quinquaud's  symptom,  already 
mentioned  in  Lecture  IV,  page  63,  is  by  no  means  infrequent  in  neurasthenics, 
while  the  phenomena  of  fibrillary  contractions  and  myokymia  described  in 
the  same  lecture  are  only  very  exceptionally  observed  here,  and  hence  should 
naturally  only  be  considered  as  neurasthenic,  after  exclusion  of  an  organic 
substratum.  In  functional  cases  they  occur  most  frequently  in  the  first  inter- 
osseus  of  the  hand. 

9.     CARDIOVASCULAR    AND    RESPIRATORY    DISTURBANCES 

The  cardiac  disturbances  in  many  neurasthenics  to  such  an  extent  occupy 
the  foreground  in  the  symptomatology  that  these  patients  frequently  turn 
at  first,  not  to  the  neurologist,  but,  thinking  that  they  have  heart  disease,  to 
the  internist.  So,  Gerhard t  affirmed  that  over  half  of  the  patients  who  came 
to  consult  him  about  heart  disease  actually  suffered  from  "neurasthenia  cordis." 
On  the  other  hand,  you  are  expressly  warned  against  making  this  diagnosis 
too  precipitately,  and  I  greatly  recommend  to  you  to  carefully  examine  the 
heart,  the  vessels,  the  urine,  etc.,  even  in  "plain  neurasthenias,"  in  order  not 
later  to  have  to  reproach  yourself  for  having  overlooked  a  myodegeneration 
of  the  heart,  a  contracted  kidney,  a  coronary  sclerosis,  etc.  The  most  im- 
portant objective  symptom  of  the  nervous  heart  is  the  quickening  of  the  pulse, 
which  may  be  permanent — analogous  to  that  which  we  have  learned  to  know 
in  Basedow's  disease, — but  which  shows  itself  usually  only  on  occasion,  by 
preference  at  night,  but  also  during  the  day  particularly  with  psychic  dis- 
turbances. In  exaggerated  cases  there  is  true  paroxysmal  tachycardia,  with 
abrupt  rise  in  the  number  of  heart  beats  to  160  to  200  per  minute. 

A  diagnostically  valuable  symptom  is,  further,  the  increase  of  lability  and 
variability  of  the  pulse-beat.  By  lability  I  understand  the  difference  between 
the  number  of  beats  under  different  conditions,  for  instance  on  change  of 
position  (lying,  sitting,  standing),  on  effort  (for  example,  mounting  upon 
a  chair  3  to  6  times,  rapidly  one  after  another,  which  corresponds  to  a  work 
of  100  to  200  meter-kilogrammes) — as  variability,  the  difference  between  the 
pulse-rate  on  different  days  (measured  as  near  as  possible  under  identical 
conditions).  In  healthy  people  pulse  lability  and  variability  are  slight;  in 
cardio-vasomotor  neurasthenia,  on  the  contrary,  exceedingly  great.     The  same 


THE    PSYCHONEUROSES  415 

tiling  applies  to  the  lability  and  variability  of  the  blood-pressure,  as  I  have 
been  able  to  determine  by  measurements  with  Gartner's  tonometer,  which  for 
the  rest  in  such  forms  is  on  the  average  abnormally  high.  While,  for  example, 
I  found  in  healthy  persons  an  average  of  95  mm  with  the  Gartner  apparatus, 
among  27  neurasthenics  with  heart  and  vascular  symptoms  I  found  the  blood- 
pressure  only  7  times  under  100,  8  times  between  100  and  115,  over  this  12 
times.  The  maximum  was  160.  Only  in  two  cases  were  the  figures  abnormally 
low,  75  and  60  mm.  To  be  placed  parallel  to  this  is  the  great  rarity  of  a 
functional  nervous  bradycardia.  Where  this  is  present  in  neurasthenics,  it 
can  often  be  found  that  there  is  a  combination  with  nicotinism. 

Irregularities  of  the  heart  action  also  belong  to  the  rare  objective  symp- 
toms of  neurasthenia.  Among  them,  true  "pulsus  inasqualis"  (allorhythmia) 
upon  a  purely  functional  basis,  that  is,  if  an  affection  of  the  heart  muscle  can  be 
certainly  excluded,  may  be  counted  a  rarity ;  somewhat  more  frequently  the 
heart  neurosis  is  accompanied  by  the  missing  of  individual  contractions,  extra- 
systoles  or  arhythmia.  In  no  way  unusual,  however,  is  the  abnormal  response 
of  the  heart  to  the  respiratory  phases,  in  that  on  inspiration  an  alteration  of 
the  pulse  in  the  sense  of  "pulsus  respiratione  intermittens"  makes  itself  more 
or  less  apparent. 

That  upon  a  purely'  nervous  basis,  variations  in  the  size  of  the  heart,  the 
so-called  "acute  dilatation  of  the  heart"  can  occur,  I  do  not  consider  proved; 
also,  in  spite  of  the  favorite  diagnosis  "nervous  heart  weakness,"  I  have  al- 
ways considered  the  finding  of  symptoms  of  stasis  (engorged  liver,  congestion 
of  the  kidney,  cyanosis  of  the  lips,  oedema,  etc.),  even  when  these  were  only 
of  slight  degree,  as  proof  of  an  organic  heart  disease,  anil  this  has  always 
proved  correct.  On  the  other  hand,  in  neurasthenics  "cardioptosis"  is  a  strik- 
ingly frequent  symptom;  if  such  patients  are  placed  upon  the  left  side  the 
apex-beat  moves  across  the  mammillary  line  outward,  and  this  "movable  heart" 
i-  also  to  be  recognized  by  percussion.  Further  the  second  sound  over  the 
aorta  and  the  pulmonary  valves  is  often  abnormally  loud.  According  to 
Oppenhebm,  upon  excitement,  besides  increased  rapidity  of  the  pulse,  a  tem- 
porary systolic  blowing  may  appear.     I  have  never  been  able  to  confirm  this. 

The    objective    heart     anomalies    just    enumerated    are    practically    always 

accompanied  by  subjective  troubles,  among  which  palpitation,  the  feeling  of 
painful  thumping  of  the  heart,  occupies  the  first  place.  Of  other  sensations, 
precordial  pressure,  which  may  increase  to  actual  "pseudo-angina  pectoris," 
comes  into  consideration.  Between  these  last,  in  which  psychogenic  compo- 
nents in  the  sense  of  "exaggerated  perception"  which  we  have  considered  in 
the  preceding  lecture  must  play  the  chief  role,  and  which  also  has  properly 
been  designated  "precordial  anxiety,"  and  the  already  mentioned  "angina 
pectoris  vaso-motoria,"  the  boundaries  are  not  always  to  be  sharply  drawn. 
An  original  symptom  of  cardiac  neurasthenia,  which  Trommer  his  described, 
you  can  hen'  ami  tin  re  confirm.  This  is  a  soiled  area  on  the  shirt-bosom  oi 
male  patients,  corresponding  to  the  position  of  the  heart,  which  has  been 
produced    by    their    constantly    placing    the    hand    upon    the    heart     region    on 

account  of  the  disagreeable  sensations  there  localized. 

Of  vasomotor  disturbances  there  an   most  frequently  to  he  found  in  neuras- 


416  LECTURE    XXVII 

thcnics  "rushes  of  blood,"  that  is,  congestions  of  the  head  with  great  local 
feeling  of  heat ;  the  ears  and  cheeks  feel  hot  and  sometimes  the  cervical  vessels 
heat  with  exaggerated  intensity.  With  the  hyperemia  of  the  head  is  contrasted 
often  the  coldness  and  lividity  of  the  hands  and  feet,  also  a  rapid  alternation 
of  reddening  and  pallor  is  peculiar  to  many  patients.  A  cooperation  of  the 
psyche  manifests  itself  in  many  cases  in  characteristic  manner;  his  tendency 
to  frequent  and  motiveless  blushing  is  well  known  to  the  patient;  he  fears  it 
("ereuthophobia")  and  this  preoccupation  is  to  blame  that  blushing  actually 
occurs  regularly  upon  meeting  unknown  persons,  in  any  sort  of  situation 
not  entirely  common.  Also  circumscribed  and  migrating  erythematous  spots 
("fliegende  roten")  are  a  frequent  symptom  in  neurasthenia.  Dermographism 
and  factitious  urticaria  occur  in  the  same  manner,  as  has  been  described  for 
Buscdoio's  disease.  But  many  neurasthenics  also  suffer  from  spontaneous  urti- 
caria, and  are  further,  as  we  already  emphasized  in  the  beginning  lecture, 
predisposed  to   different  vasomotor  neuroses. 

Much  more  rare  than  the  vasomotor-cardiac,  are  respiratory  symptoms 
in  neurasthenics.  In  spite  of  the  certain  relations  of  bronchial  asthma  "to 
nervous  influences  there  are  actually  among  the  great  horde  of  neurasthenics 
strikingly  few  typical  asthmatics.  What  is  designated  by  such  patients  as 
asthma  is  generally  only  a  "nervous  tachypnoea"  sometimes  running  parallel 
with  the  tachycardia. 

10.     ANOMALIES    OF    SECRETION 

The  secretory  anomalies  of  neurasthenia  are  somewhat  less  frequent  than 
the  cardio-vasomotor.  Most  widespread  is  readily  the  tendency  to  excessive 
outbreaks  of  sweat,  general  or  localized  ( forehead,  hands,  feet)  ;  in  the  second 
place  are  to  be  mentioned  anomalies  of  the  gastric  secretion,  among  which 
hyperacidity  is  the  rule,  only  exceptionally  hypacidity,  or  even  anacidity. 
The  occurrence  of  an  acid  gastrorrhoea,  in  attacks,  is  called  "paroxysmal 
gastroxynsis."  Increased  salivation  and  polyuria  are  rare,  exceedingly  fre- 
quent, on  the  contrary,  the  symptom  denominated,  little  justifiably,  "phos- 
phaturia,"  a  precipitate  of  the  earthy  phosphates  (calcium  and  magnesium) 
soluble  in  acids,  present  in  freshly  passed  urine,  or  occurring  when  it  is 
warmed.  Without  a  trace  of  proof  this  precipitate  has  been  improperly 
interpreted  as  a  sign  of  increased  decomposition  of  phosphorus  containing 
nerve  substance  (lecithin,  protagon).  This  view,  however,  may  be  considered 
as  aside  from  the  subject,  so  long  as  it  is  not  supported  by  exact  experiments 
on  metabolism  carried  out  under  all  precautions  upon  such  phosphaturic  neu- 
rasthenics, and  there  are  no  such  at  present.  With  the  simple  determination 
of  phosphates  in  the  urine  nothing  can  be  decided;  satisfactory  conclusions 
can  only  be  expected  from  the  exact  determination  of  the  phosphorus  intake 
and  its  excretion  by  the  neurasthenic  during  a  given  time.  At  the  present 
time  I  consider  it  probable  that  simple  alteration  of  the  conditions  of  solu- 
bility in  the  urine  are  to  be  held  responsible  for  the  so-called  neurasthenic 
phosphaturia.  These  are  perhaps  due  to  abnormalities  in  the  production  of 
acids  in  the  body.     Finally,  is  to  be  mentioned  the  "mucous  colic"  or  "pseudo- 


THE    PSYCHONEUROSES  417 

membranous  colitis,"  in  which,  in  attacks  with  severe  abdominal  pain,  tube- 
like formations  of  mucus  and  fibrin  are  discharged.  This  disease,  a  secretory 
neurosis  of  the  colon,  is  observed  particularly  in  psychoneurotic  patients 
(mainly  females)  ;  the  popular  designation,  "nerve  mucus,"  customary  in 
many  places,  shows  sharp  observation  on  the  part  of  the  laity. 


LECTURE  XXVIII 
The  Psychoneuroses 

B.      Neurasthenia    (Continued) 

Gentlemen:  We  have  now  brought  to  an  end  the  analytical  consideration 
of  the  manifold  subjective  and  objective  semiology  of  acquired  neurasthenia, 
begun  in  the  last  lecture,  and  from  now  on  we  will  direct  our  attention  more  to 
the  grouping  of  symptoms  than  to  single  symptoms.  It  is  self-evident,  and  I  have 
sufficiently  emphasized  it,  that  all  these  disease  symptoms  cannot  be  present  in 
every  neurasthenic ;  rather  must  an  exceedingly  great  variety  of  clinical  pic- 
tures arise,  according  to  the  special  prominence  of  this  or  that  anomaly  and 
the  absence  or  recession  of  other  disturbances.  The  necessity  for  systematiza- 
tion  has  given  rise  to  the  separation  of  the  disease  according  to  such  so-called 
"■localizations"  of  the  neurasthenias   into  special  varieties. 

So,  many  neurologists  speak  of  cerebral  neurasthenia  or  encephalasthenia 
when  the  troubles  of  the  patient  consist  chiefly  in  pressure  in  the  head,  tin- 
nitus aurium,  vertigo,  disturbances  of  sleep,  irritability,  etc.  As  hyperalgetic 
neurasthenia  has  been  denominated,  that  form  in  which  pain  in  the  back  and 
disagreeable  and  painful  sensations  in  the  limbs  constitute  the  chief  complaint. 
Such  disease  pictures,  however,  in  which  palpitation  of  the  heart,  congestions, 
tachycardia,  fleeting  erythema,  dermographism,  etc.,  predominate,  are  classed 
as  cardio-vasomotor  neurasthenia.  These  three  varieties,  however,  show  so 
many  transition  forms  and  combinations  that  their  separation  must  be  con- 
sidered quite  an  artificial  one.  A  much  more  independent  position  must,  on 
the  contrary,  be  assigned  to  those  varieties  of  neurasthenia  which  are  denom- 
inated nervous  dyspepsia  and  sexual  neurasthenia,  hence  you  shall  have  a 
connected  exposition  of  these  before  we  turn  to  the  etiology  and  pathogenesis 
of  neurasthenic  conditions. 

NERVOUS    DYSPEPSIA 

Here  disturbances  on  the  part  of  the  digestive  organs  to  such  an  extent 
occupy  the  foreground  that  it  is  the  stomach  specialists,  and  not  the  neurolo- 
gists, who  have  contributed  mainly  to  our  knowledge  of  this  exceedingly 
widespread  form  of  disease. 

The  average  picture  of  nervous  dyspepsia,  which  naturally  presents  the 
most  varied  degrees  of  intensity,  is  about  the  following:  The  patients  com- 
plain of  a  frequent  burdensome  feeling  of  pressure  that  either  comes  on  after 
meals  or  is  independent  of  taking  food.     The  qualitative  character  of  the  food 

418 


THE    PSYCHONEUROSES  419 

is  in  remarkable  manner  often  a  matter  of  entire  indifference,  or,  indeed,  there 
is  the  paradoxical  phenomenon  that  heavy,  fat.  acid  foods,  etc.,  are  best 
borne;  hence,  sometimes  the  peculiar  "regimes"  which  many  such  patients 
lay  down  for  themselves,  and  to  which  they  hold  fast  with  great  pedantry 
and  obstinacy. 

Precipitate  change  of  the  disease  picture  is  a  frequent  occurrence;  often 
we  seek  in  vain  for  an  adequate  cause  for  the  beginning  of  the  dyspeptic 
trouble,  very  frequently,  however,  the  influence  of  psychic  factors  is  un- 
mistakable; an  anger,  an  excitement,  sometimes  a  ridiculously  slight  annoy- 
ance, has  '"fallen  on  the  stomach"  of  the  patient,  and  only  with  the  passing 
away  of  the  mental  annoyance  the  troublesome  symptoms  disappear.  Fre- 
quent accompanying  symptoms  of  stomach  pressure  arc  sour  or  stale  eruc- 
tations, anomalies  of  the  stomach  chemism  (usually  in  the  sense  of  a  hyper- 
acidity (see  above,  page  416),  heartburn,  regurgitation).  The  appetite  is 
usually  capricious,  anorexia  and  buliminia  can  alternate  with  one  another. 
Quite  characteristic  is  a  painfully  perceived  feeling  of  hunger  which  usually 
occurs  on  an  empty  stomach  (particularly  at  night  or  upon  awakening  in 
the  morning),  and  has  been  called  by  Boas  "gastralgokenosis."  The  tongue 
often  looks  quite  normal;  usually,  however,  it  is  slightly  coated;  also  the 
intestinal  functions  are  in  very  many  cases  disturbed;  there  is  most  frequently 
atonic  constipation  {KwssmauTs  "torpor  peristalticus"),  sometimes  spastic 
constipation;  that  for  the  rest  the  combination  of  atony  in  the  proximal  and 
spasm  in  the  distal  divisions  of  the  colon  occurs,  Stierlin  has  of  late  recog- 
nized roentgenological^-.  Among  the  spastically  constipated  dyspeptics,  the 
disposition  to  occasional  paroxysms  of  mucous  colic  is  most  widespread.  More 
frequent,  indeed,  than  the  last  so  exceedingly  characteristic  disturbance,  there 
are  in  nervous  dyspepsia,  in  consequence  of  peristaltic  intestinal  unrest,  diar- 
rhoeas. Such  "nervous  diarrhoeas"  are  often  abruptly  set  up  by  an  emotion, 
a  fright,  etc.  Very  frequent  is  distressing  pressure  to  empty  the  bowels, 
which  usually  occurs  at  an  inconvenient  time   (in   the  theater,   for  example). 

A  by  result  of  the  intestinal  atony,  found  particularly  distressing  by  tin' 
patient,  is,  finally,  flatulence;  distention  making  itself  apparent  during  the 
night,  is  often  designated  as  the  direct  cause'  of  sleep  disturbances.  In  spite 
of  these  manifold  digestive  disturbances  the  condition  of  nutrition  of  the 
patient  in  nervous  dyspepsia  does  not  usually  sutler  to  an  appreciable  de- 
gree. There  are  found  among  them,  indeed,  people  of  blooming  appearance 
to  whom  may  be  applicable  the  exclamation  of  the  visitor  of  Beard's,  who, 
after   a   look    at    his    waiting-room    filled    with    neurasthenics,   cried    out,    "Your 

patients  are,  indeed,  regular  giants." 

It  is  otherwise,  however,  with  the  (in  general,  rare)  severe  eases  of  nervous 
dyspepsia,  since  here  there  may  hi'  extreme  emaciation,  indeed,  real  marasmus. 

In  such  cases  the  gastric  troubles  have  taken  on  a   chronic  character,' no!    remit 

ting  in  anv  appreciable  extent.  Loss  of  appetite  predominates.  Along  with 
the  slight  desire  for  nourishment  the  continued  anxiety  as  to  supposed  dietary 
errors  leads  the  patient  to  limit  his  hill  of  fare  more  and  more,  and  to 
undertake  actual  fast  cures  in  which  In-  is  often  not  .aware  of  the  insufficiency 

of  his   nutrition.      In   one   of  my    patients,   for  example,   who.  although    reduced 


420  LECTURE    XXVIII 

to  a  skeleton,  affirmed  that  he  abundantly  nourished  himself,  it  appeared  from 
the  investigation  of  his  metabolism  by  Jaquet  that  he,  in  his  hypochondriacal 
anxiety  about  abdominal  distress,  had  actually  reduced  his  ration  to  1,850 
calories  a  day  (an  average  of  3  days).  (As  a  comparison  it  may  be  recalled 
that  in  a  woman  lying  in  hysterical  sleep,  the  daily  minimum  needed  was  reck- 
oned by  Sonden  and  Tigerstedt  at  1,680  calories,  while  Voit  gives  the  normal 
food  value  for  adults  at  moderate  labor  at  about  2,750  calories.)  The  loss 
of  strength  in  severe  cases  of  nervous  dyspepsia  can  be  increased  in  that  there 
is  not  only  eructation  and  regurgitation,  as  in  the  slighter  forms,  but  there 
may  be  actual  vomiting.  Since,  further,  the  feeling  of  pressure  in  the  stomach 
sometimes  increases  to  more  or  less  severe  pain,  the  differential  diagnosis  of 
such  cases  from  ulcer  or  carcinoma  of  the  stomach  is  often  only  possible 
after  long  observation  with  the  aid  of  Roentgenological,  chemical  and  micro- 
scopical examination  of  the  stomach  motility  and  stomach  contents. 

SEXUAL    NEURASTHENIA 

To  the  preponderant  role  which  sexuality  plays  in  the  life  of  the  individual 
and  its  manifold  relationships  to  the  most  varied  expressions  of  the  psyche, 
corresponds  the  great  extent  of  those  forms  of  neurasthenia  in  which  the  whole 
disease  picture  revolves  about  disturbances  of  the  sexual  function.  If  the 
symptoms  here  are  exclusively  those  to  which  organic  basis  is  to  be  denied, 
nevertheless  their  appearance  in  material  diseases  of  the  male  or  female  sexual 
organs  (in  the  sense  of  a  simple  combination)  is  a  very  frequent  occurrence. 
This  is  explainable,  as  we  will  assume  in  advance  of  our  discussion  of  the 
etiology  and  pathogenesis  of  neurasthenia,  in  unconstrained  manner  from  the 
fact  that  diseases  of  the  genital  organs,  particularly  venereal  affections,  to 
a  special  degree  favor  hypochondriacal  self-observation  and  brooding.  When 
discussing  the  treatment  of  neurasthenia  I  will  shortly  impress  upon  you  the 
necessity  of  educating  the  patients  to  look  upon  their  troubles  with  as  much 
equanimity  as  possible,  even  to  disregard  them.  Allow  me  to  warn  you  against 
too  close  inquiry  as  to  sexual  anomalies  which  may  furnish  still  more  encour- 
agement to  reflection  on  things  which  without  this  already  dominate  too  much 
the  thoughts  of  the  patient.  Here  the  necessity  of  finding  out  the  cause,  with 
the  conscientious  physician,  must  give  way  to  the  principle  "non  nocere"  (do 
no  harm). 

For  the  rest,  the  patients  almost  always  acquire  such  confidence  in  a 
tactful,  reserved  medical  adviser  that  they  spread  out  before  him  voluntarily 
a  sufficiently  detailed  sketch  of  the  disturbances  in  question.  These  last  can 
be  quite  satisfactorily  arranged  under  Beard's  definition  of  "irritable  weak- 
ness." Indeed,  abnormal  sexual  irritability  and  exhaustability  can  sometimes 
be  differentiated  as  a  first  and  second  disease  stage;  more  frequently,  how- 
ever, symptoms  of  both  categories  occur  actually  along  with  one  another. 
In  our  consideration  we  will  for  clearness  hold  fast  to  this  division. 

The  increased  irritability  first  manifests  itself  in  that  the  sexuality  makes 
itself  evident  in  a  manner  inadequate  to  the  constitution  of  the  patient.  Erotic 
thoughts  press  upon  ,the  psyche   in   such  predominant   fashion  as   can   often 


THE    PSYCHOXEUROSES  421 

find  no  satisfaction  within  the  sphere  of  the  attainable,  and  develop  into  sexual 
phantasies  and  waking  dreams.  Upon  the  same  basis  arise,  also,  the  most 
varied  sexual  perversions.  As  physical  expressions  of  the  hyperaphrodisia 
are  to  be  mentioned,  in  men  excessive  and  continued  erections  which,  particu- 
larly at  night,  reach  distressing  intensity  and  may  lead  to  an  agrypnia  which, 
on  account  of  the  ever-present  danger  of  masturbation,  is  particularly  exhaust- 
ing. Still  more  serious  than  this  last  is  psychic  "onanism,"  orgasm  intention- 
ally provoked  by  means  of  erotic  phantasies.  To  be  mentioned  further  are  the 
excessively  frequent  (sometimes,  indeed,  nightly  or  several  times  in  a  night) 
nocturnal  pollutions.  If  these  are  to  be  regarded  as  a  morbid  manifestation, 
when  they  occur  in  periods  of  sexual  abstinence,  only  from  excessive  fre- 
quency and  from  the  psychic  and  physical  depression  which  follows  them, 
diurnal  pollutions  are  to  be  considered  absolutely  and  without  exception  as  a 
pathological  occurrence.  They  occur,  nevertheless,  only  in  the  most  severe 
forms  of  sexual  neurasthenia.  From  the  increased  sexual  irritability,  the 
carrying  out  of  the  sexual  act  is  usually  interfered  with,  often  impossible; 
namely  there  is  premature  ejaculation.  x\s  irritative  symptoms  in  sexual 
neurasthenia  in  females  are  to  be  mentioned:  vaginismus  (intercourse  pre- 
vented), pruritus  of  the  genitals  (a  starting-point  for  masturbatory  practices) 
and  pollutions,  discharges  of  mucus  from  the  glands  about  the  vaginal  en- 
trance (Bartholin's  glands,  etc.)  occurring  with  orgasm,  in  half-sleeping  con- 
ditions. 

As  a  symptom  of  weakness  is  to  be  mentioned  the  great  exhaustion  which 
even  with  active  sexual  desire  follows  coitus  as  well  as  pollutions.  Sexual 
weakness  in  the  male  occurs  most  markedly,  however,  in  the  form  of  impotence 
which  here,  in  contradistinction  to  the  organic  form  (about  which  we  learned 
in  connection  with  tabes,  for  example),  is  to  be  denominated  psychic.  If 
here,  insufficient  response  of  the  nerves  of  the  sexual  organs  to  erotic  stimuli 
is  often  at  fault  (anaphrodisia),  as  a  rule,  the  chief  blame  is  to  be  ascribed 
to  fear  of  failure,  lack  of  self-confidence,  etc.,  for  this  chief  complaint  of  male 
sexual    neurasthenics. 

Still  higher  grades  of  sexual  weakness  are  characterized  by  the  substitu- 
tion for  pollutions  of  the  discharge  of  semen  while  awake  and  without  orgasm. 
This  spermatorrhoea  occurs  upon  urination  and  defecation  (micturition  and 
defecation    spermatorrhoea).       In    women    sexual    weakness    manifests    itself    ill 

absolute  frigidity. 

The  near  relationships  between  the  genital  and  the  uropoetic  apparatus, 
renders  it  easily  understandable  thai  the  majority  of  sexual  neurasthenics  also 
complain  of  urinary  troubles,  namely  of  desire  to  urinate  at  inconvenient  times. 
pollakiuria,  dysuria  and  strangury.  Particularly  characteristic  is  the  symptom 
which  has  been  called  '•urinary  stuttering."  Tin  patienl  during  micturition 
must   repeatedly  stop,  and  so  empties  his  bladder  in  small  quantities  at  a  time. 

Finally,  without  exception,  genera]  neurasthenic  symptoms  manifest  them 
selves  in  disturbing  fashion,  often  favored  l>\  the  fear  of  threatened  disease 
of  the   spinal   cord.      The   belief  on    the    part    of   the   public   that    sexual    neiiras- 

thenia  is  the  precursor  of  "consumption  of  the  spinal  cord"  ( tabes)  maintains 
itself  with   ineradicable  obstinacy,  since  it    is   intentionally  supported   by   the 


422  LECTURE    XXVIII 

quacks  and  charlatans.  The  patients  show  a  more  or  less  severe  depression, 
occasionally  increasing  to  suicidal  impulsion,  are  misanthropic,  unable  to  ap- 
ply themselves  to  any  work,  lose  all  interest,  complain  of  pain  in  the  back, 
headache,  etc. 

Pathogenesis  and  Etiology 

Since  now  we  have  obtained  a  sufficient  insight  into  the  semiology  of  neu- 
rasthenic conditions,  having  used  as  examples  the  ordinary  acquired  neuras- 
thenia as  well  as  two  of  its  varieties  practically  important,  nervous  dyspepsia 
and  sexual  neurasthenia,  we  must  enter  more  fully  into  the  question  of  the 
nature  and  the  occurrence  of  such  and  similar  symptom-complexes. 

Here  I  would  refer  to  my  remarks  already  made  in  Lecture  XXVII,  on  the 
question  as  to  whether  the  psychoneuroses  are  to  be  counted  among  the  heredi- 
tary or  the  acquired  diseases.  We  saw  there,  that  between  the  endogenic  and 
the  exogenic  factors  forming  the  basis  of  the  individual  case  there  exists  a 
reciprocal  relation,  so  that  on  the  one  hand  under  the  influence  of  powerful 
external  factors,  even  a  normally  constituted  nervous  system  is  affected,  on  the 
other,  a  neuropathic  disposition  brought  into  the  world  can  lead  to  the  develop- 
ment of  pathological  conditions  even  from  the  ordinary  stresses  of  life. 

For  proper  estimation  of  the  individual  neurasthenic  disease  picture,  it  is 
now  of  prime  importance  to  take  into  consideration  whether  an  endogenic  or 
degenerative  factor  is  present  or  absent.  It  is  not  the  least  service  of  Charcot 
that  he  first  perceived  the  necessity  for  separating  what  he  called  true,  and 
what  hereditary,  neurasthenia.  In  order  to  assign  to  both  etiological  varieties 
the  same  rank  within  the  limits  of  Beard's  disease,  indeed,  we  had  better  strike 
out  the  epithet  "true"  and  use  for  it  "acquired"  or  "accidental"  neurasthenia, 
in  contradistinction  to  "congenital"  or  "constitutional  neurasthenia."  That 
these  two  varieties  prognostically  are  to  be  estimated  quite  dissimilarly,  and 
therapeutically  are  to  be  managed  in  an  entirely  different  way,  does  not  need 
to  be  specially  emphasized.  That,  however,  this  principle  of  classification  is  a 
correct  one,  appears  from  the  circumstances  that  in  their  symptomatology, 
also,  as  you  will  see  directly,  the  accidental  and  the  constitutional  cases  show 
differences,  whose  importance  leaves  far  behind  all  that  formerly  justified  the, 
attempt  at  a  topographico-symptoniatological  classification  (see  above,  page 
418). 

Let  us  turn  next  to  the  etiology  of  the  accidental  form,  which  includes  al- 
ways recognizable  and  well-characterized  influences,  often  affects,  acutely,  a 
previously  entirely  normal  organism,  and  in  which  neuropathic  heredity  plays 
no  role  worth  speaking  of  (see  page  407).  When  we  ask  the  patient  himself 
what  causes  he  holds  responsible  for  his  nervosity,  we  will  hear  him  complain 
either  of  strokes  of  fate  suffered,  of  agitations,  distress  and  care,  mental  over- 
exertion, business  troubles, — he  accuses  himself  of  sexual  excesses,  or  finally, 
he  dates  his  nervous  weakness  back  to  a  physical  disease  of  acute  or  chronic 
course,  from  which  he  has  suffered.  When  we  now  search  through  this  rather 
varied  list  of  causal  factors,  after  a  common  criterion,  it  may  be  seductive  to 
regard  the  factor  of  exhaustion  usually  as  such.  So  Mbbius  would  consider 
acquired  neurasthenia  as  a  variety  of  chronic  exhaustion,  its  individual  symp- 


THE    PSYCHOXEUROSES  423 

toms  as  potentiated  exaggerated  symptoms  of  the  physiological  fatigue  process, 
developed  into  a  permanent  condition.  This  pathogenetic  conception,  however, 
does  decided  violence  to  the  facts  and  hence,  has  even  in  neurological  circles, 
been  able  to  establish  no  support.  Of  course,  there  is  a  chronic  fatigue,  an 
exhaustion  become  a  permanent  condition  ("defatigatio")  ;  we  observe  it  after 
long  lying  in  bed  with  fevers,  in  the  undernourished,  among  the  women  of  the 
proletariat  affected  by  hard  work  and  numerous  labors,  after  exhausting  ma- 
neuvers, after  excessive  feats  of  strength  in  various  sports,  but  we  miss  in  this 
the  most  important  physical  and  psychic  stigmata  of  Beard's  psychoneurosis, 
and  the  picture  of  simple  fatigue  is  rather  that  of  a  very  general  relaxation 
and  prostration,  which  usually  disappears  leaving  no  symptoms,  upon  rest  and 
full  nourishment,  without  medical  aid. 

For  the  development  of  an  acquired  neurasthenia,  something  more  is  needed 
than  for  that  of  a  chronic  exhaustion,  not  the  excess  of  stimuli  that  press  upon 
the  nervous  system,  but  their  qualitative  nature  gives  the  result  its  marked 
emotional  coloring. 

An  example:  A  business  man  attributes  his  severe  neurasthenia  to  the 
heaping  up  of  the  duties  of  his  calling;  and  still  he  admits  that  some  years  back 
he  passed  over  a  period  of  much  more  severe,  even  excessive  exertion,  almost 
like  child's  play.  The  explanation  is  furnished  us  by  the  fact  that  he  at  that 
time  was  in  a  position  of  less  responsihility,  but  in  the  meantime  had  moved 
up  to  a  post  of  heavy  responsibility.  The  robust  nervous  system  over  which 
the  affect-free  exertion  passed  without  trace,  gave  way  under  that  marked 
by  strong  affect.  Also  in  the  wide-spread  "examination  neurasthenia"  the 
anxious  tension,  not  the  forced  work,  is  chiefly  to  be  blamed. 

Now  there  are  indeed  persons  enough  who  under  such  circumstances  do  not 
fall  victims  to  neurasthenia,  and  then'  is  no  doubt  that  certain  "tempera- 
ments" are  more  exposed  to  the  acquisition  of  a  neurasthenia  than  others. 
The  person  conscientious  from  the  start,  strict  with  himself,  may  be  considered 
as  predisposed;  the  one  of  sentimental  nature  and  soft  heart,  not  less  so;  none 
more  so  than  the  ambitious  person  whose  mental  work  can  never  develop  with- 
out affect,  but  through  stimulated  expectation,  or  painful  undeception  ex- 
periences  a  continuous  emotional   coloring. 

So  we  understand  also,  why  neurasthenia  grew  first  in  North  America  to 
be  a  disease  of  the  masses;  in  the  last  decades,  however,  it  has  overflown  into 
the  civilized  countries  of  the  Old  World:  parallel  with  the  haste  and  unrest 
which  modern  methods  of  communication  and  the  acquisitions  of  technology   in 

all  branches  of  human  activity  have  liberated,  in  all  nations  the  tendency  to 

continually  more  complicated  methods  of  life  has  developed.  The  movements 
of  trade,  the  relations  between 'production  and  consumption,  have  become  eon 
tinuallv  more  complicated,  so  that  to  speak  with  the  national  economist  Biicher, 
'•The  existence  and  work  of  every  individual  is  interlaced  more  and  more  with 
the  existence  and  work  of  many  others.'*  In  all  departments  of  business  life, 
we  gee  more  and  more  dependence  upon  numerous  unaccountable  factors  enter, 
a  feeling  of  uncertainty  which  is  continually  increased  by  tin-  constantly  more 
acute  competition.  Along  with  these  specifically  increased  cares  of  occupa- 
tion, those  conducting  different  enterprises  hear  more  and  more  the  burden  ol 


424  LECTURE    XXVIII 

crushing  responsibility.  The  measure  of  responsibility  has,  however,  also  in- 
creased for  the  subordinates  in  industrial  and  commercial  enterprises.  The 
middle  class  is  engaged  in  a  hard  and  desperate  struggle  upon  two  fronts. 
The  psyche  of  the  working  man  is  in  continued  tension  through  the  sharpening 
of  the  social  contrasts,  through  class  struggle,  through  the  danger  to  life  of 
the  mechanical  occupations.  Also  for  him  the  danger  of  crises  is  a  continually 
threatening  one;  the  hours  of  labor  are  shorter,  but  the  work  has  become  more 
intensive  and  intellectual.  In  short,  if  the  "American  nervousness*'  of  Beard 
has  also  mastered  modern  Europe,  this  has  its  basis  in  those  phenomena  which 
have  been  designated  as  the  "Americanization"  of  our  social  conditions. 

Also  in  the  cases  where,  following  bodily  diseases,  a  symptomatolcgicallv 
typical  neurasthenia  (not  as  more  frequently  a  simple  exhaustion)  is  acquired, 
the  affective  factor  is  never  absent.  No  wonder  that  among  these  diseases, 
just  those  which  give  occasion  for  distressing  self-reproaches,  or  to  anxious 
fears  for  the  future,  far  excell  all  others  in  importance  and  frequency;  namely, 
the  venereal  affections.  Also  in  the  sexual  neurasthenia  caused  through  excesses 
or  errors,  there  manifest  themselves,  as  the  most  important  etiological  agents, 
remorse,  shame,  but  particularly  the  fear  of  evil  results.  That  this  fear  is 
encouraged  by  quacks  and  medical  persons  fallen  into  devious  ways,  by  bro- 
chures, prospectuses,  etc.,  which  have  as  their  end  to  draw  into  their  nets  as 
many  victims  as  possible,  I  have  already  emphasized.  One  of  the  most  favored 
tricks  of  these  people  consists  in  representing  such  normal  things  as  the  oc- 
casional occurrence  of  pollution  in  sexual  abstinence,  and  one  testicle  hanging 
lower  than  the  other,  as  the  consequences  of  youthful  sins,  and  the  precursors 
of  incurable  marasmus,  and  in  suggesting  in  this  manner  to  the  readers  of  their 
literature,  sexual  neurasthenic  troubles,  for  which  then  they  assert  that  they 
possess  the  only  cure.  For  the  origin  of  severe  dyspepsia  sometimes  improper 
medical  treatment  is  at  fault,  as  Dejcrine  has  forcibly  emphasized  quite  re- 
cently. As  a  prophylactic,  one  should  refrain  in  neurasthenics  from  bringing 
into  action  for  slight  digestive  disturbances,  which,  however,  are  greatly  ex- 
aggerated by  the  patient,  the  imposing  apparatus  of  complicated  bills  of 
fare,  frequent  washing  out  of  the  stomach,  water  cures,  etc.,  but  should  on  the 
contrary  seek  to  represent  to  the  patient  his  troubles  as  unimportant  and  fleet- 
ing symptoms  and  should  avoid  overtreatment. 

Special  importance  in  this  era  of  social  legislation  has  been  acquired  by 
"accident  neurasthenia,"  which  often  in  little  satisfactory  manner  has  been 
brought  together  with  accident  hysteria,  the  fright  neuroses,  commotion  psy- 
choses, etc.,  as  "traumatic  neurosis."  Accident  neurasthenia  develops  from 
the  abnormal  mental  condition  into  which  the  injured  person  falls  as  a  con- 
sequence of  the  accident,  especially  when  this  has  affected  the  "noble  parts" 
(the  head,  spine,  region  of  the  heart).  The  fear  of  permanent  injury  to  health 
or  economically,  especially  about  the  effect  upon  his  ability  to  work  and  earn 
money,  puts  the  patient  into  a  state  of  chronic  emotional  unrest  which  in  spite 
of  undisturbed  recovery  from  the  material  results  of  the  accident,  may  develop 
into  the  severest  neurasthenia.  A  particularly  injurious  influence  is  exercised 
heri'  by  preoccupation  with  regard  to  the  question  of  damages,  which  the 
compensation  and  accident  insurance  laws  of  the  different  civilized  states  cause 


THE    PSYCHONEUROSES  425 

the  injured  person.  Fear  that  he  will  receive  too  little  indemnity  keeps  him  in 
continued  tension  :  it  more  and  more  impresses  itself  upon  his  mind  that  high 
damages  are  due  him,  and  there  is  anxiety  lest  this  be  cut  down  or  withheld 
from  him,  either  carelessly  or  malignantly,  or  that  his  troubles  will  be  estimated 
too  lightly. 

The  system  of  paying  a  monthly  allowance  customary  in  Germany,  for  in- 
stance, greatly  encourages  the  formation  of  such  "avaricious  concepts,"  and 
indeed  the  continued  "struggle  for  pension''  furnishes  ever  fresh  material  for 
accident  neurasthenia.  Much  more  rational  is  our  Swiss  system  of  cash  settle- 
ment, in  which  the  injured  person  after  being  paid  the  sum  awarded  by  agree- 
ment or  through  a  decision  of  Court,  is  as  greatly  interested  on  the  ground  of 
health,  as  financially  in  regaining  as  soon  as  possible  his  full  capacity  to 
work.  In  what  manner  the  estimation  of  what  compensation  should  be  paid 
to  the  traumatic  neurasthenic  is  to  be  estimated,  cannot  be  rigidly  laid  down. 
A  first  principle  is,  however,  that  a  permanent  impairment  of  working  capacity 
must  not  be  assumed  upon  the  basis  of  a  traumatic  neurasthenia  (naturally  the 
most  exact  observation  must  have  permitted  the  exclusion  of  symptoms  organi- 
cally caused).  As  an  expert,  I  take  the  stand  that  according  to  the  severity 
of  the  neurasthenia  present  (as  to  which  the  objective  symptoms  should  be 
decisive),  it  must  be  considered  that  the  disabilities  should  be  estimated  as, 
likely  to  extend  over  from  six  months  to  at  most  three  years.  My  after- 
histories,  in  agreement  with  those  of  Nageli  and  other  observers,  prove  the 
correctness  of  this  view. 

The  closing  portion  of  one  of  my  reports,  for  example,  reads  as  follows : 

"The  cure  of  the  troubles  is  dependent  upon  the  earliest  removal  possible 
of  the  question  of  compensation.  As  long  as  tins  is  pending,  in  spite  of  all 
the  efforts  of  the  physician,  the  subject  will  be  exposed  to  emotional  dis- 
turbances which  will  furnish  conditions  favoring  the  continuation  of  the  trau- 
matic neurosis  present.  If,  however.  Mr.  X  realize-,  that  the  question  of  com- 
pensation is  definitely  settled,  his  troubles  will  gradually  disappear  anil  he 
will  regain  his  ability  to  work. 

"In  consideration  1,  of  the  kind  and  manner  and  of  the  intensity  of  the 
present  troubles  of  the  subject  (vertigo,  headache,  hypochondriacal  depres- 
sion); '2,  of  his  occupation  as  a  carpenter  ( which  requires  frequent  bending 
over  and  in  which  vertigo  is  very  disturbing)  ;  and  .'5,  of  experiences  acquired 
in  similar  cases,  I  would  recommend  to  the  Court  a  compensation  upon  the 
basis  of  the  assumption  of  a  diminution  of  earning  capacity  from  •_'•">  to  33 
per  cent,  (luring  2  years.  Though  the  reduction  of  working  capacity  is 
greater  at  present,  it  is  nevertheless  to  he  assumed  that  after  definite  adjudi 
cation,  this  will  diminish  progressively  and  at  latest  will  have  disappeared  at 
the  end  of  the  second  year." 

Quite  otherwise  than  in  the  acquired  forms  are  the  etiologico-pathogenetic 
relationships  in  constitutional  neurasthenias.  Tin-  degenerative  factor  mani 
feats  itself  equally  from  the  anamnesis  and  from  the  semiology,  now  in  unmis 
takable  intensity,  now  in  more  obscure  forms,  sometimes  indeed,  only  as  a  sug- 
gestion. For  neuropathic  heredity,  in  contradistinction  to  the  acquired  case., 
we  seldom  seek  in  vain,  also  it   i.  usually   furnished  by  numerous  and  seven 


426  LECTURE    XXVIII 

forms  of  disease  in  the  ascendants  or  in  the  collateral  branches  of  the  family. 
These  disease  forms  belong  only  in  the  minority  of  the  cases,  under  the  division 
of  similar  heredity.  More  frequently  (and  in  the  more  severe  cases)  there 
are  in  the  parents,  brothers  and  sisters,  not  neurasthenic  symptom-complexes, 
but  more  severe  neuropathies,  hysteria,  psychoses,  epilepsy,  so  that  the  "de- 
generative neurasthenic"  in  contradistinction  to  the  victims  of  a  reinforced 
pathological  heredity  (see  Lecture  VII,  page  197)  often  appears  to  us  as  the 
relatively  least  injured  member  of  an  in  general  much  more  severely  degenerated 
family.  Often,  also,  separated  still  further  from  homomorphous  heredity,  we 
find  common  among  the  progenitors,  affections  injurious  to  the  germ  in  general, 
as  alcoholism,  syphilis,  tuberculosis,  more  rarely  diabetes,  perhaps  also  the 
uric  acid  diathesis,  if  we  follow  the  statements  of  French  and  English  authors, 
since  with  us  the  rarity  of  true  gout  does  not  permit  the  formation  of  a 
definite  opinion  in  this  direction.  Finally,  consanguinity  of  the  parents  is  to 
be  considered  here. 

There  are  now,  however,  also  neurasthenias  of  the  most  severe  degenerative 
character  in  which  we  can  find  no  hereditary  factor.  In  general  we  must  ex- 
pect to  form  a  decisive  conclusion  as  to  the  constitutional  factors  from  the 
study  of  the  patient  himself.  And  hence  we  will  take  up  now  the  previously 
unconsidered  clinical  peculiarities  of  these  degenerative  cases. 

CONSTITUTIONAL    NEURASTHENIAS 

The  three  chief  criteria  of  the  cases  falling  under  this  head  are:  1,  the 
occasional  presence  of  somatic  stigmata  of  degeneration;  2,  beginning  of  the 
disease  manifestations  in  early  or  earliest  childhood ;  3,  certain  anomalies  to 
be  considered  as  psychic  stigmata  of  degeneration. 

The  first  point  we  have  considered  at  length  in  our  introduction  to  psy- 
choneuroses  (Lecture  XXVII),  and  only  need  to  refer  to  it  to-day  to  refresh 
your  memory.  Only  it  must  be  expressly  emphasized  that  such  morphological 
anomalies,  which  are  entirely  foreign  to  acquired  neurasthenia,  are  not  very 
frequent  also  in  the  constitutional  form. 

Much  more  frequently,  however,  in  any  event  in  the  majority  of  the  cases, 
there  is  a  history  of  the  so-called  congenital  neurasthenia  or  that  of  child- 
hood. We  find  that  already  in  school,  or  even  before  this,  in  the  nursery,  the 
patient  is  characterized  by  abnormal  irritability,  motiveless  outbursts  of 
anger;  that  he  now  misanthropically  and  discontentedly  isolates  himself  from 
contemporaries,  again  manifests  a  restless  motor  impulsion,  that  he  suffers 
from  stuttering,  or  for  years,  often  until  puberty,  from  nocturnal  enuresis ; 
that  he  has  masturbated  long  before  the  age  of  puberty.  Important,  also,  is 
the  history  that  the  patient  as  a  child  suffered  from  night  terrors,  those  pe- 
culiar attacks  of  frightened  waking  out  of  sleep  with  momentary  confusion  and 
loud  screaming,  which  even  then,  when  they  are  referred  to  nasal  polypi, 
adenoid  vegetations  of  the  pharynx  or  to  intestinal  worms,  indicate  an  ab- 
normal reflex  excitability  of  the  brain.  Milder  fright  conditions  are  more 
frequent;   fear  of  darkness,  of  the  whistle   of  a   locomotive,   of  being  alone: 


THE    PSYCHONEUROSES  427 

further  peculiar  habits,  eating  paper,  forming  senseless  words,  crying  out  on 
certain  occasions,  grimacing  during  serious  situations,  etc. 

Over  these  manifold  expressions,  which  it  is  often  not  at  all  easy  to  dis- 
cover anamnestically,  the  psychic  stigmata  which  so  often  as  phobias  and  im- 
perative conceptions  dominate  the  clinical  picture  of  constitutional  neuras- 
thenia in  adults,  cast  their  shadow. 

As  best  known  and  perhaps  most  frequent  paradigm  of  the  phobias,  West- 
phal's  "agoraphobia"  (fear  of  open  spaces)  is  to  be  mentioned.  This  is  an 
overpowering  feeling  of  anxiety  which  always  affects  the  patient  when  he  has 
to  traverse  an  open  space.  A  feeling  of  anxiety,  which  expresses  itself  in  the 
countenance,  is  accompanied  by  pallor  and  palpitation  of  the  heart,  the  break- 
ing out  of  a  cold  sweat,  and  the  arrest  of  the  salivary  secretion.  A  feeling  of 
anxiety  in  which  the  person  affected  has  the  sensation  that  he  is  about  to  sink 
through  the  ground,  that  his  legs  are  paralyzed.  A  feeling  of  anxiety  that  in 
severe  cases  actually  compels  the  individual  to  turn  about,  to  creep  along  the 
houses,  or  to  solicit  the  attendance  of  a  passer-by,  although  he  is  entirely 
aware  of  the  lack  of  motive  and  absurdity  of  his  phobia.  Endless  is  the  list 
of  other  phobias  which  we  meet  as  a  frequent  or  occasional  occurrence,  in  the 
picture  of  constitutional  neurasthenia,  and  to  which  an  endless  number  of 
foreign  words  have  been  applied;  the  gephyrophobia  already  described  in  the 
works  of  Hippocrates,  that  is  the  fear  of  passing  over  a  bridge;  siderodromo- 
phobia, the  fear  of  traveling  on  a  railroad  train;  claustrophobia,  the  fear  of 
being  pent  up  in  a  narrow  space.  Excessively  variable  are  the  special  mani- 
festations of  the  so-called  "situation  anxiety,"  which,  for  example,  affects  the 
barber  when  he  is  about  to  shave,  the  preacher  when  he  is  about  to  mount  the 
pulpit,  etc. ;  all  manifestations  which  are  strictly  separable  from  the  vague 
anxiety  of  the  accidental  neurasthenic. 

Closely  related  to  the  conditions  of  anxiety  are  imperative  conceptions 
(obsessions),  from  which  the  constitutional  neurasthenics  so  often  suffer. 
"Conceptions  which  do  not  arise  by  way  of  associations,"  so  Oppcnheim  de- 
fines them,  "but  appear  spontaneously  and  forcibly  press  into  the  circle  of 
ideas,  so  that  they  cannot  be  banished  from  them  although  the  individual  con- 
siders them  something  foreign,  not  belonging  to  the  mental  ego."  The  most 
frequent  paradigm  of  these  disturbances  is  the  "Folic  du  doute"  ("doubting 
mania")  studied  by  Griesinger,  Legrand  da  Saulle,  and  Faint  long  before 
Beard. 

This  consists  in  questions  which  continually  and  without  motive  press  upon 
the  mind,  as  "Why  have  I  said  this  and  not  that?"  or  doubts  which  cause  even 
the  mosi  indifferent  acts  to  take  on  a  painful  character  and  are  often  com- 
bined with  anxious  concepts,  ''Would  it  lie  better  to  lake  this  journey  or 
not?"  ".May  I  not  be  the  victim  of  a  railroad  accident?"  or  "Maj  mil  some 
body  break  into  the  house  (hiring  my  absence?"  "Did  I  really  put  a  stamp  on 

the  letter    just   mailed?"  "Have   I    not    forgotten   to   shut    tin-  door  of  my  house1** 

"Have  I  not  forgotten   to  turn  out   the  gas?"     Such  doubts  appear  compul 

BOrily    upon    the    most    indifferent    occasions    (they    can   occur   as    episodes    even 

within  physiological  limits)   and  are,  besides,  firsi   disproportionately  strong, 

and,  second,  so  obstinate  that  they  do  not  allow  the  patient  to  rest,  although 


428  LEGTURE    XXVIII 

(and  partly  because)  there  is  complete  insight  for  the  senselessness  and  patho- 
logical character  of  these  manifestations,  a  phenomenon  which  is  easily  differ- 
entiated from  the  general  lack  of  decision  of  the  accidentally  neurasthenic. 

More  severe  symptoms  are  arithmomania,  the  compulsion  to  count  the  win- 
dows of  a  house,  the  lanterns  in  a  street,  the  flowers  in  the  pattern  of  a  carpet, 
onomatomania,  the  compulsion  to  call  to  mind  certain  forgotten  proper  names, 
the  "deli  re  du  toucher,"  the  obsession  that  a  defilement  or  infection  must  be 
connected  with  every  touching  of  a  strange  person  or  object,  etc. 

As  to  the  psychical  mechanism  of  the  peculiar  phenomena  of  the  phobias 
and  obsessions,  we  can  with  good  conscience  repeat  "Ignoramus,"  since  the 
attempt  of  Sigmund  Freud  to  explain  imperative  conceptions  as  in  general 
symbolical  from  certain  "displaced"  psychic  complexes  of  erotic  nature  can- 
not be  brought  into  accord  with  the  facts ;  also  I  consider  the  sharp  separation 
between  an  anxiety  neurosis  and  a  compulsion  neurosis  undertaken  by  him  as 
impracticable. 

Much  more  does  it  recommend  itself  to  denominate  both  forms  with  their 
transitions  just  as  frequently  found,  with  Janet  and  Raymond,  as  "Psychas- 
thenia."  Now  "psychic  powerlessness"  (and  this  is  indeed  caused  by  the  giv- 
ing way  without  resistance  under  hyper-quantivalent  ideas  in  spite  of  com- 
plete insight),  impresses  a  characteristic  stamp  upon  the  whole  clinical  picture. 
This  does  not  indeed  indicate  that  somatic  signs  of  disease  are  absent — still 
they  play  a  much  smaller  role  than  in  acquired  neurasthenia.  It  seems  to  me 
deserving  of  emphasis  that  pressure  in  the  head  and  sleeplessness  never  reach 
a  very  high  degree,  indeed,  can  be  entirely  absent.  Among  the  objective  symp- 
toms which  we  mentioned  for  the  acquired  form,  only  the  increase  of  reflexes 
and  the  cardio-vasculur  lability  usually  manifest  themselves  with  marked  in- 
tensity, sometimes  also  vibrating  tremor.  Of  other  somatic  phenomena  the 
not  altogether  rare  occurrence  of  "tics"  (see  Lecture  IV,  pages  67  and  72), 
indeed  deserves  mention. 

The  conceptions  psychasthenia  and  constitutional  neurasthenia  would  agree 
with  one  another  if  it  were  not  that  a  considerable  contingent  of  cases  which 
in  their  history  prove  themselves  certainly  constitutional,  are  entirely  wanting 
in  the  phobic  and  obsessive  element,  and  approach  the  picture  of  accidental 
neurasthenia.  Indeed,  they  then  usually  bear  a  chronic  character,  the  time 
of  their  development  cannot  be  precisely  determined  and  they  have  a  less 
stormy  symptomatology.  In  spite  of  this,  just  these  cases  are  to  be  con- 
sidered much  more  serious  and  therapeutically  much  less  infruenceable — which 
of  course  applies  also  to  the  psychasthenias. 

Diffcren  Hal  Diagnosis 

Now  before  we  pass  over  to  the  so  important  treatment  of  neurasthenic 
conditions,  it  must  not  be  neglected  to  point  out  the,  unfortunately  all  too  fre- 
quent, serious  diagnostic  and  practical  misconceptions  which  arise  from  the 
extension  of  the  conception  neurasthenia,  to  conditions  which  have  with  it 
nothing  in  common  but  a  more  or  less  extended  semiological  analogy.  We 
should  hold  to  the  rule,  only  to  make  the  diagnosis  neurasthenia,  by  exclusion. 


THE    PSYCHONEUROSES  429 

At  first  it  must  be  considered  that  incipient  phthisis,  diabetes  mellitus,  chronic 
alcoholism,  nicotinism,  morphinism  and  cocainism  may  produce  similar  symp- 
toms. The  same  remark  applies  to  certain  organic  brain  diseases  whose 
"pseudo-neurasthenic"  initial  stage  we  have  already  described  at  length :  de- 
mentia paralytica,  and  cerebral  arteriosclerosis.  Dementia  praecox  should  also 
be  thought  of  here :  indeed,  for  example,  it  has  been  shown  by  Mile.  Pascal  that 
of  75  patients  sent  to  the  Insane  Asylum,  Ville-Evrard,  with  dementia  praecox 
(of  both  the  simple  as  well  of  the  paranoid,  hebephrenic  and  catatonic  varie- 
ties) not  less  than  32  had  first  been  wrongly  diagnosed  and  treated  for  neuras- 
thenia, an  error  which  may  have  serious  results  for  the  patient,  his  family 
and  society.  Against  such  serious  errors  only  careful  psychological  investi- 
gation into  the  mental  condition  of  the  patient  will  protect  us.  It  will  be 
noticed  that  in  the  neurastheniform  prodromal  stage  of  dementia  praecox — in 
contradistinction  to  true  neurasthenia — emotional  indifference  is  the  basal 
element  of  the  psychic  syndrome, — the  hypochondriac  ideas  are  characterized 
liv  their  variability  and  their  trifling  character,  concern  themselves  prepon- 
derantly about  the  physical  condition  of  the  health  and  are  emitted  with  little 
affective  accompaniment  (emotional  dulling).  Notice  also,  peculiarities,  odd 
demeanor,  motiveless  laughing;  negativism  in  the  form  of  systematic  opposi- 
tion, and  a  hard-headedness  alternating  with  childish  docility  and  suggestibil- 
ity; "psychographic  disturbances,"  an  excess  of  pretentious  word-forms  used 
b}'  preference,  bombastic  and  inflated  style  further  appear.  Quite  as  frequently 
the  confusion  of  neurasthenia  with  cyclothymia,  that  is,  witli  the  "formes 
frustes"  of  circular  melancholia  occurs.  This  happens  in  patients  usually  of 
the  female  sex,  who  at  more  or  less  regular  periods — for  example,  every  Spring 
or  every  Fall,  become  relaxed,  without  energy,  and  inclined  to  tears,  complain  of 
dyspeptic  troubles,  pressure  in  the  head  and  similar  things,  and  usually  suffer 
from  marked  agrypnia.  As  definite  indications  of  their  relationship  to  melan- 
cholia, besides  the  periodicity  to  be  mentioned  are,  the  regular  occurrence  of 
self-reproach  and  depressive  ideas  ("I  cannot  preside  over  my  house,  am  a 
burden  to  my  family!  Oh!  what  a  lazy  ami  unenergetic  person  I  am!"  etc.), 
and  that  in  the  great  majority  of  cases  there  is  an  alternation  of  morning 
exacerbations  and  evening  remissions.  That  the  formes  frustes  of  Basedow's 
disease  usually  sail  under  the  colors  of  neurasthenia  cannot  be  denied;  also  one 
must  he  on  his  guard  againsl  confusing  this  lasl  with  beginning  Addison's 
disease. 

Treatment 

The   interest    which   of  late  has  been   specially  directed    to   the   |is ychoneiiroses 

in  general  and  to  neurasthenia  in  particular,  has  fortunately  redounded  also  to 
the  advantage  of  the  therapeutic  side  of  the  problem.  Proceeding  to  discuss 
these  matters  I  shall  chiefly  keep  in  mind  those  methods  which  can  be  carried 
out  outside  of  a  hospital  or  sanitarium;  since  mosl  of  you  will  meet  your 
neurasthenics  in   private   practice. 

In  every  rational  treatment  of  neurasthenic  conditions  the  Leading  posi 
tion  must  be  assigned  to  those  measures  for  which  the  word  "psychotherapy" 
has  been  coined.     That  this  expression  has  become  quite  a  commonplace,  must 


430  LECTURE    XXVIII 

be  admitted.  Nevertheless,  it  has  one  great  advantage  in  that  it  shows  us  in 
striking  manner,  how  great  a  change  our  therapeutic  measures  for  psycho- 
neurotic affections  have  experienced  of  late.  Here  the  service  of  the  path- 
finding  work  of  Paul  Dubois  cannot  be  enough  emphasized.  Although  even 
before  him,  consciously  or  unconsciously,  every  really  good  physician  practiced 
mental  treatment,  and  Ottomar  liosenbach  is  to  be  considered  as  his  prede- 
cessor in  this  method  of  cure  (the  so-called  "dialectic  psychotherapy"),  it  is 
only  through  the  efforts  of  Dubois  that  the  recognition  of  how  predominant 
a  position  is  to  be  attributed  to  psychic  influences  in  the  circle  of  therapeutic 
agents  has  become  generally  realized,  and  how  astonishing  results  it  is  per- 
mitted to  obtain  by  its  systematic  application.  Further,  he  has  made  it  clear 
to  us  that  psychotherapy  has  its  technique  and  its  rules,  like  all  other  methods, 
and  that  to  be  applied  intelligently  and  systematically  it  presumes  in  the 
physician  a  certain  makeup  besides  practice  and  experience.  These  require- 
ments can  never  be  replaced  by  theoretical  study,  also  you  should  not  expect 
from  me  more  than  some  suggestions  and  indications  toward  developing  your 
own  psychotherapeutic  system.  According  to  the  personality  of  the  thera- 
peutist his  methods  will  have  to  bring  into  play  the  factors  of  educational 
influence,  explanation  of  facts  and  authoritative  intervention  in  quite  varied 
combinations,  to  estimate  whose  relative  value  would  be  a  sterile  pedantry. 
Rather  are  they  to  be  judged  by  the  effects  produced.  That,  however,  the 
personality  of  the  patient  must  have  a  decisive  influence  upon  the  choice  of  the 
psychotherapeutic  method,  is  self-apparent;  every  word  that  the  physician 
addresses  to  a  neurasthenic  exercises  upon  him  a  psychic  action,  and  hence 
must  be  adapted  to  his  peculiarities. 

So,  there  are  individuals  who  are  entirely  competent  to  realize  that  they 
must  suffer  throughout  life  from  certain  troubles,  if  it  is  only  permitted  to 
the  physician  to  convince  them  that  these  symptoms  do  not  place  in  question 
either  their  lives  or  their  working  capacity ;  other  neurasthenics,  on  the  con- 
trary, cannot  bring  themselves  into  such  a  condition  of  stoicism,  and  by  the 
(direct  communication  of  such  a  prognosis  are  reduced  to  despair  and  driven 
deeper  into  their  psychoneurosis.  It  is  not  given  to  every  one  to  react  to  the 
appeal  to  duty,  altruistic  feeling,  etc.,  in  the  sense  of  correcting  the  egocentric 
view  of  the  world  peculiar  to  many  neurasthenics.  Occasionally  we  appeal 
directly  to  self-love — so  doing  must  of  course  be  carefully  considered  in  ad- 
vance— "If  you  do  not  carry  out  my  recommendations  and  put  aside  the  in- 
jurious factors  pointed  out  to  you,  you  are  liable  to  become  still  wTorse."  In 
short,  a  great  measure  of  diplomatic  skill  is  necessary,  in  properly  estimating 
how  the  patient  is  best  to  be  readied,  corresponding  to  his  psychological  in- 
dividuality. This  insight  into  the  nature  of  the  patient  we  must  endeavor  to 
obtain,  particularly  from  the  manner  in  which  he  describes  his  troubles  to  us. 
Since  this  is  usually  done  with  extraordinary  detail  and  verbosity,  we  have,  as 
a  rule,  only  too  much  time  to  become  acquainted  with  the  mentality  of  the 
individual.  Naturally,  as  I  have  already  emphasized  in  the  last  lecture,  it 
must  be  our  endeavor  not  to  allow  the  history  to  be  extended  indefinitely,  since 
we  cannot  devote  all  our  time  to  a  single  patient.  But  we  need  not  cut  off  a 
neurasthenic  by  saying  to  him,  "What  you  wish  to  tell  me  further  is  unim- 


THE    PSYCHONEUROSES  431 

portant ;  I  know  enough,"  since  by  so  doing  we  would  irreparably  lose  his  con- 
fidence. On  this  account  one  must  learn,  by  skillful  introduction  of  questions, 
to  lead  the  patient  away  from  unimportant  matters  and  to  keep  his  descrip- 
tions within  reasonable  bounds.  The  neurasthenic  must,  however,  gain  the  im- 
pression from  the  conduct  of  the  physician  while  taking  the  history,  that  his 
complaints  are  sufficiently  considered  and  are  not  taken  "with  a  shrug  of  the 
shoulders."  Also  the  physical  examination  must  be  careful  and  thorough, 
since  in  this  way  we  procure  a  basis  for  convincing  strength  in  the  reassuring 
advice  which  is  to  be  imparted  to  the  patient. 

Removal  of  the  hypochondriacal  ideas  is  important  above  everything  else. 
To  this  end  I  do  not  hesitate,  in  the  case  of  intelligent  and  educated  patients, 
to  support  my  word  by  clinical  proof  and  explain  to  them,  in  a  simple  but 
scientific  way.  why  their  heart  troubles  are  to  be  considered  functional  and 
cannot  be  organic,  in  what  way  their  abnormal  sensations  are  produced;  with 
the  hemoglobinometer  I  demonstrate  that  the  suspected  anemia  is  not  present; 
by  reading  selected  paragraphs  from  Band,  Dubois,  etc.,  I  show  that  phe- 
nomena which  greatly  frighten  the  patient  do  not  represent  anything  but 
typical  neurasthenic  symptoms;  that  phobias  and  obsessions  are  not  at  all 
precursors  of  mental  darkening,  that  many  views  spread  through  inferior  sexual 
literature  which  hold  the  sexual  neurasthenic  under  their  ban,  are  fabulous,  etc. 
A  wide  and  thankful  field  for  explanation  and  reassurance.  In  this  short 
discussion  of  the  nature  and  symptoms  of  neurasthenia,  I  would  only  advise 
you  to  carefully  avoid  one  thing,  the  word  "imagination,"  which  almost  always 
makes  such  patients  restive,  while  "autosuggestion"  can  perhaps  be  risked. 

With  explanation  to  the  patient  of  the  nature  and  genesis  of  his  troubles, 
the  matter  is  indeed  finished  in  only  very  few  cases.  Usually  teaching  self- 
control,  consideration  of  the  subjective  disease  symptoms  as  a  "negligible 
quantity,"  training  into  a  rational  method  of  occupation,  correction  of  ex- 
aggerated sensitiveness  to  the  annoyances  of  life,  etc.,  requires  quite  high 
qualities  and  psychotherapeutic  skill  on  the  part  of  the  physician.  It  is 
usually  specially  difficult  to  talk  a  psychically  impotent  person  back  into  his 
lost  self-confidence,  by  far  most  troublesome,  however  (unfortunately  often 
aKo  most  thankless),  to  influence  anxious  and  imperative  conditions  through 
logic.  Here,  as  there,  it  is  ill  general  to  be  recommended,  at  first  to  advise  I  lie 
patient  as  far  as  possible  to  avoid  for  a  long  time  the  "critical  situations"  (for 
example,  cohabitation,  crossing  bridges,  etc.  Through  this,  the  memory  of 
former  ill  results  and  attacks  of  anxiety  becomes  gradually  less  marked,  and 
the  inhibitions  which  oppose  themselves  to  rational  psychotherapy  are  grad 
ually  reduced.  Further,  a  great  number  of  phobias  and  obsessions  prove  them 
selves  accessible  to  psychic  influence  only  after  there  has  been  a  change  ol 
surroundings  as  radical  as  possible;  hence,  treatmenl  in  sanitariums  and  asy- 
lums comes  frequently  into  question  in  such  cases.  For  the  rest,  however,  I 
certainly  agree  with  Edinger,  when  he  warns  against  being  too  ready  to  order 

institution  treatment.  The  slighter  cases  of  neurasthenia  are  really  often 
better  suited   when   the  changes   in   surroundings   are   procured    in   a    [ess    radical 

manner,  as  in  the  form  of  a  sojourn  iii  the  country,  or  a  sea  voyage,  provided 

that,  while  so  doing,  enough  new  impressions  are  furnished   fin    the  uiiiiil  to  keep 


432  LECTURE    XXVIII 

it  from  the  observation  of  self.  Even  the  prescription  of  such  a  cure  acts  often 
encouragingly  upon  patients  who  had  feared  to  be  sent  to  a  sanitarium.  A 
disadvantage  of  a  sanitarium  is  often  that  the  neurasthenic  patients  mutually 
lament  about  their  troubles  to  one  another,  which  hardly  acts  in  the  sense  of 
an  "abstraction."  In  neurasthenic  conditions  shall  psychotherapy  push  en- 
tirely into  the  background  other  methods  of  treatment  (formerly  incorrectly 
occupying  the  foreground),  or  has  each  of  these  its  justification  along  with 
the  other?  In  this  question  I  would  occupy  an  eclectic  stand-point  and  would 
epitomize  the  matter  as  follows : 

That  the  psychic  abnormalities  of  the  neurasthenic  cannot  be  directly  in- 
fiuenced  by  dietetic  rules  or  by  prescriptions  is  self-evident,  and  here  psycho- 
therapy in  the  direction  which  was  sketched  above  has  uncontested  results. 
Nevertheless,  in  the  state  of  mind  of  the  accidental  neurasthenic  the  primary 
is  not  always  easily  to  be  differentiated  from  the  secondary,  namely  what  is 
irritable  weakness  of  the  brain  functions  from  what  is  the  natural  result  of  a 
frequently  quite  painful  feeling  of  general  loss  of  strength  anel  prostration. 
The  last,  however,  can  be  influenced  by  somatic  measures  of  treatment.  To 
the  subjective  disturbances  in  whose  estimation  the  seconelary  ideogenic  element 
is  difficult  to  differentiate  from  the  results  of  primary  injury,  belong  headache 
and  pain  in  the  back,  loss  of  sleep,  loss  of  appetite,  vertigo,  etc.  Here,  also, 
psychotherapy  belongs  in  the  foreground;  as  further  curative  factors,  how- 
ever, physical  and  dietetic  methods  can  be  applied,  and  properly  applied,  they 
give  excellent  results.  Only  in  the  second  place,  however,  should  treatment 
of  these  subjective  disturbances  by  drugs  come  into  application,  since  in 
no  other  instance  is  there  greater  danger  of  the  establishment  by  the  super- 
ficial therapeutist  of  a  '"therapy  of  little  symptoms."  The  following  principles 
should  prevail:  "Limitation  of  the  indications  as  much  as  possible — the  even- 
tual application  of  drugs  is  permissible  only  as  a  therapeutic  compromise  and 
by  reason  of  opportunity,  and  in  any  case  as  rarely  and  as  temporarily  as 
possible."  This  applies  namely,  to  the  different  "headache  remedies"  from 
the  group  of  the  antipyretics  and  for  hypnotics  proper. 

The  better  one  understands  how  to  carry  on  the  general  treatment  of 
neurasthenia,  the  more  rarely  will  he  have  to  resort  to  remedies  of  this  class, 
if  only  temporarily,  it  should  always  be  considered  as  a  forced  position.  Not 
only  does  the  objection  to  this  convenient  method  of  treatment  with  sympto- 
matic drugs  rest  upon  the  danger  of  habit  formation,  but  also  in  that  it 
directly  opposes  a  rational  psychotherapy.  Everything  is  in  bringing  the 
patient  primarily  to  the  conviction  that  all  his  individual  complaints  are  not 
diseases  in  themselves,  but  represent  the  outcome  of  a  unique  abnormalization 
of  the  whole  nervous  system.  If,  however,  we  force  these  by  too  active  thera- 
peutic consideration  into  the  foreground  of  the  treatment,  we  make  him  doubt- 
ful as  to  our  assurances  that  his  "everlasting  pain  and  misery  so  thousand- 
fold" is  to  be  cured  from  one  point.  It  is  somewhat  different,  however,  with 
the  manifold  somatic  visceral  disturbances,  which  we  have  enumerated  as  ob- 
jective symptoms  of  neurasthenia.  These  disease  manifestations  (for  ex- 
ample, abnormal  variability  of  the  pulse  and  of  the  blood  pressure,  factitious 
urticaria,   hvperidrosis,  fine  tremor,   tachycardia,  hyperacidity,   etc.)    do   not 


THE    PSYCHONEUROSES  433 

permit  themselves  to  be  absolutely  subjected  to  a  primordial  mental  alteration 
as  in  hysteria,  in  which  the  symptoms  manifesting  themselves  in  the  territory 
of  somatic  or  sympathetic  n,erves  are  brought  to  pass  in  a  roundabout  way 
through  the  conception.  Rather  is  it  here  plainly  the  coordination  of  irritable 
weakness  both  in  the  psychic  and  in  the  physical  sphere,  so  that  in  neurasthenia 
we  can  recognize  a  general  neurosis  in  the  fullest  sense  of  the  word.  The 
somatic  visceral  disturbances  of  neurasthenia  have  hence  a  claim  to  a  treat- 
ment to  a  certain  degree  autonomous,  to  be  begun  along  with  psvchotherapy 
and  based  upon  a  rational  empirical  basis.  The  disappearance  of  these  symp- 
toms will  be  an  indication  of  its  efficacy,  that  is,  a  reduction  of  the  reflex  and 
mechanical  irritability,  and  return  to  normal  secretory  conditions,  the  quieting 
of  the  heart,  etc.,  and  since  we  can  accomplish  these  actually  by  physical, 
dietetic  and  medicinal  agents,  I  cannot  decline  these  methods.  Presuming  that 
with  the  removal  of  the  causal  factors,  an  accidental  neurasthenia  can  be  cured, 
a  constitutional,  decidedly  improved,  by  psychotherapy  alone,  nevertheless,  in 
my  experience  our  task  is  greatly  lightened  in  most  cases  by  a  combined  pro- 
cedure. 

No  drug  is  so  often  given  to  the  neurasthenic  as  the  alkaline  bromides ;  to 
many  physicians  inclined  to  routine,  ordering  this  drug  follows  almost  as  a 
matter  of  course  the  diagnosis  neurasthenia.  There  is,  however,  no  point  in 
administering  bromides  to  relaxed  neurasthenics  without  any  marked  symptoms 
of  excitation.  At  most  a  short  course  of  bromide  (with  daily  doses  up  to 
about  3  grins  of  KBr)  can  be  occasionally  recommended  for  such  patients  in 
whom  irritative  symptoms  dominate  the  clinical  picture,  while  weakness  occu- 
pies the  background;  for  example,  in  threatened  predominance  of  conditions 
of  anxiety,  in  disturbance  of  the  always  labile  psychic  equilibrium  from  some 
annoyance  small  or  great.  Very  dilute  solutions  are  best  borne  (10  grammes 
(3iiss.)  of  the  salt  in  150  cc  (§v)  of  aq.  menth.  pip.,  of  which  8  tablespoonful 
can  be  taken  in  a  glass  of  water).  If  to  this  mixture  0.3  to  O.-i  (gr  iv  to  vi) 
codein  phosphate  is  added,  the  sedative  action  is  increased. 

A  further  quite  useful  sedative  is  Indian  hemp,  in  the  prolonged  adminis- 
tration of  which  very  small  doses  suffice.  It  can  also  very  well  be  combined  with 
tonic  remedies;  for  example,  1  have  recommended  the  following  combination 
under  the  name  of  pilulffi  cannahin;e  composite: 

I J      Quinini  sulphat 1.0   (gr.  xv) 

Acid,  arseniosi 0.00 — 0.1    (gr.    %o   to   1/4) 

Extract,   cannabis   ind 0.45   (gr.  <>:,i) 

Extr.  et  pulv.  rad.  valerian  q.  s.  ut   f.   pil.  xxx. 

S.     One  pill  every  evening. 

A  course  of  arsenic  is  sometimes  above  everything  a  curative  factor  in  thin, 
chronically  fatigued  neurasthenics  having  little  appetite.  Large  doses  are 
practically  never  desirable;  as  a  rule,  in  long  administration  a  daily  dose  of 
().()()•_'  to  0.003  (gr  ':■.,,  to  Yzo)  of  arsenious  acid  or  0.2  to  0.8  (  nyiii  to  ITRv) 
of  Fowler's  solution,  is  sufficient.  The  last,  combined  with  tine,  nucis  vom.,  is 
usually  well  borne.     .More  convenient  are  the  Asiastic  pills,  of  which  one  can 


434  LECTURE    XXVIII 

be  given  daily  after  the  evening  meal  (Acid,  arsenics,  0.2;  pip.  nigri,  5.0; 
sach.  alb.,  rad.  alth.  aa.  .3.0,  M.  f.  pil.  No.  C).  Where  exceptionally  a  very 
sensitive  stomach  refuses  the  arsenic  preparations,  I  administer  them  sub- 
cutaneously  in  the  form  of  sodium  cacodylate  in  (sterile  ampoules  of  0.05). 
Where  there  is  definite  anemia,  iron  is  called  for.  Very  popular  are  Erb's 
''Tonic  Pills":  Ferr.  lactat.,  extract,  cinchon.,  aquos,  aa  4.0  (3i).  Extract. 
nucis.  vom.,  1.0  (gr.  xv)  ;  extract,  gentian,  q.  s.  ut  f.  pil.  No.  C.  S.  2  pills 
t.  i.  d.  after  meals. 

Where  iron  does  not  appear  indicated,  cinchona  and  nux  vomica  are  ordered 
in  the  form  of  drops  (tinct.  nuc.  vom.,  5.0;  tinct.  cinchon.  comp.,  10.0;  M.  S. 
30  drops  t.  i.  d.  before  meals,  in  water).  This  prescription  is  useful  in  in- 
testinal atony  and  other  dyspeptic  troubles. 

A  drug  in  which  tonic  and  sedative  properties  are  united  is  valerian,  an 
indication  for  which  is  furnished  particularly  by  the  cardio-vascular  disturb- 
ances. A  systematic  course  of  the  infusion,  prepared  in  the  cold,  has  proved 
most  efficient.  Directions  for  its  use  will  be  found  under  the  treatment  of 
Basedow's  disease  (see  Lecture  XXIII,  page  353).  Where  the  valerian  tea  is 
refused,  the  extract  can  be  substituted  for  it,  for  example:  Ty .  Extract. 
valerian.  10.0  (oiiss)  ;  extract,  hyoscyam.,  zinc.  oxid.  pur.,  aii  5.0  (gr  lxxv). 
M.  Fiat  Pil.  No.  C.  S.  1  pill  three  or  four  times  a  day.  Phosphoric  medica- 
tion, when  it  is  indicated,  namely  in  thin  but  full  blooded  neurasthenics,  is  to 
be  carried  out  as  recommended  under  Basedow's  disease  (under  treatment  with 
sodium  phosphate,  or  calcium  glycero-phosphate,  there  is  often  satisfactory 
gain  in  weight  and  improvement  in  the  general  condition),  further,  in  neuroses 
of  the  stomach  and,  finally,  in  nervous  irritative  conditions  of  the  sense  organs, 
particularly  in  tinnitus  aurium. 

Also  with  regard  to  one  further  point  we  can  refer  to  what  was  said  under 
Basedow's  disease  in  Lecture  XXIII.  The  ovo-lacto-vegetarian  regime  can 
act  exceedingly  favorably  in  neurasthenics  with  marked  circulatory  lability; 
quite  as  satisfactory  is  sometimes  the  effect  of  such  change  of  diet  in  nervous 
dyspepsia,  in  which  the  gastric  anomalies  of  secretion  are  favorably  influenced 
by  the  relative  lack  of  irritation  of  the  mild  meatless,  or  meat-poor  diet. 
Finally,  this  is  the  most  harmless  method  of  combating  the  chronic  intestinal 
sluggishness  so  frequent  in  nervous  people,  particularly  when  an  increase  in 
the  content  of  cellulose  is  provided  for  by  the  free  use  of  fruit,  Graham  bread, 
etc.,  and  in  obstinate  cases,  %  liter  of  warm  physiological  salt  solution  is 
given  in  the  morning  on  an  empty  stomach.  The  intestinal  fermentation  and 
the  distention  by  which  the  atonic  intestine  of  the  neurasthenic  is  so  often 
troubled,  are  decidedly  reduced.  Where  there  is  anemia,  naturally  in  the  regula- 
tion of  diet  its  content  in  iron  should  be  taken  into  consideration  (spinach, 
carrots,  yolk  of  egg,  oats,  asparagus,  strawberries,  Graham  bread).  In  cer- 
tain cases,  particularly  in  cardio-vasomotor  neurasthenia,  alcohol  is  best  entirely 
forbidden  ;  under  all  conditions,  however,  it  is  only  to  be  permitted  in  small 
quantities  and  well  diluted.  Strong  coffee  is  to  be  forbidden,  at  most,  that  well 
diluted  with  milk  is  permitted.  Tea  appears  to.  be  less  injurious,  but  also  is  to  be 
permitted  only  in  small  amounts.  With  regard  to  tobacco,  it  is  to  be  forbidden 
in  the  cardiac  and  vasomotor  forms  of  neurasthenia;  otherwise,  however,  we 


THE    PSYCHONEUROSES  435 

should,  as  a  rule,  be  satisfied  with  restricting  its  use  within  moderate  bounds. 
Indeed,  we  should  consider  the  agreeable  feeling  which  it  procures  for  the  pa- 
tient as  an  ally,  from  a  psychotherapeutic  standpoint,  since  it  raises  his  spirits. 

A  simple  dietetic  prescription  which  well  suits  most  patients  with  sense 
of  pressure  in  the  head  and  dizziness,  is  to  allow  them  to  take  some  slight 
refreshment  every  hour  in  the  day  so  that  the  stomach  is  almost  never  quite 
empty.*  It  is  astonishing  how  rapidly  almost  every  one  becomes  accus- 
tomed to  this  regime  and  how  advantageous  it  is  found.  In  cases  in  which 
the  head  troubles  mentioned  above,  together  with  weakness  and  prostration, 
make  themselves  apparent  immediately  after  rising,  breakfast  had  better  be 
taken  in  bed  about  one  hour  before  getting  up.  This  simple  dietary  measure 
can  be  denominated  "fractional  overnutrition" ;  in  ambulatory  treatment  and 
in  slight  cases,  it  can  accomplish  what  is  sought  in  severe  cases  by  the  "Weir 
Mitchell  rest  cure,"  which  in  any  case  can  only  rarely  be  carried  out  at  home, 
and  for  which  in  general  a  sojourn  in  a  hospital  is  necessary. f  The  marked 
taking  on  of  flesh,  which  is  attained  in  such  cures  by  the  gradual  training  to 
continually  more  frequent  and  continually  more  nutritious  meals  and  aided  by 
the  large  amount  of  physical  rest,  often  acts  as  a  marked  sedative  to  the  whole 
nervous  system. 

A  certain  comfortable  laziness  gradually  comes  over  those  subjected  to 
this  "cure,"  and  the  proverb  "Plenus  venter  non  studet  libenter"  gradually  finds 
application  in  the  matter  of  constantly  studying  himself  and  his  woes  by 
such  a  patient.  Besides  this,  the  isolation  with  which  the  Weir  Mitchell  cure  is 
united,  gradually  prepares  the  way   fur  psychotherapy. 

The  physical  curative  measures  which  may  he  of  value  to  the  neurasthenic 
can  be  only  briefly  mentioned.  With  warm  full  baths  and  half-baths,  air- 
baths,  hot  foot-baths,  alternating  foot-baths  and  cool  spongings,  we  can  succeed 
win  rever  baths  are  found  advantageous  and  we  do  not  need  complicated  hvdro- 
therapeutic  apparatus.  The  foot-baths  are  particularly  appropriate  in  com- 
bating the  congestions  and  tinnitus  aurium,  the  full-baths  and  half-baths  for 
quieting  the  patient  at  night,  the  rubbings  and  the  air-baths  Cor  general  tonic 

treatment.  With  cold-Water  procedures  ami  sun  baths  for  which  tile  laity 
have  such  a  great  fondness  we  are  more  likely  to  do  harm  than  to  aid.  Also 
the  wide-spread  idea  that  the  practice  of  different  athletic  sports  is  a  panacea 
for  nervosity  must  he  decidedly  opposed.  It  is  well  to  lay  down  for  the  pa- 
tients, who  seek  recovery  in  the  country,  rational  directions  with  regard  to 
walks,  periods  of  rest,  reclining  in  the  open  air,  etc.,  as  otherwise  we  risk  hav- 
ing  them  comi  hack  in  a  seriously  exhausted  condition.  Finally,  I  would  recom- 
mend to  von  to  take  to  In-art, the  reminder  of  Heard:  "Every  ease  of  neuras- 
thenia is  a  study  in  itself;  no  two  eases  are  just  alike  If  two  eases  arc  treated 
alike  from  start    to  finish   it    is  probable  thai   one  of  them  has  hern   wi 

treated." 

*  For  example,  milk,  cakes,  figs,  prunes,  chocolate  tabli  I  .  1 te. 

.  Doctor  Mitchell  always  preferred  carrying  ou1   the  "real  cure"  in  special  privuti 
away  from  the  hospital. — Translator. 


LECTURE  XXIX 
The  Psychoneuroses 

C.     Hysteria 

In  contradistinction  to  neurasthenia,  which,  as  we  saw,  has  only  been  iso- 
lated nosologically  and  named  in  recent  times,  in  its  sister  neurosis  hysteria, 
we  have  to  do  with  a  very  ancient  disease  conception.  That  the  striking  symp- 
toms of  the  disease  were  brought  into  connection  with  supposed  disturbances 
of  the  functions  of  the  uterus,  has,  as  is  known,  found  expression  in  the  name 
for  the  affection  ( baripa  =  the  womb).  Hippocrates  regarded  the  hysterical 
phenomena  to  a  certain  extent  as  abstinence  symptoms  on  the  part  of  the 
organ  withdrawn  from  its  natural  function ;  the  disease  appeared  most  fre- 
quently in  old  maids  or  in  women  early  widowed,  to  whom  the  advice  to  have 
relations  with  men  as  soon  as  possible  must  be  given;  "since  if  they  become 
pregnant,  they  will  be  cured."  Particularly  fantastic  is  the  pathogenetic  con- 
ception attributed  to  Timaus,  who  imagined  that  the  unsatisfied  uterus  wan- 
dered restlessly  about,  like  a  rutting  animal  ("animal  liberorum  procreandorum 
appetens"="an  animal  desirous  of  begetting  children"),  throughout  the  body, 
and  in  this  way  set  up  the  hysterical  symptoms.  That  this  grotesque  view  has 
been  retained  to  this  day  among  the  country  people  of  our  neighboring  Alsace, 
deserves  mention :  we  occasionally  hear  from  a  peasant  woman  troubled  about 
her  daughter,  the  expression  "her  womb  is  trying  to  get  out  of  her  throat." 

Further,  you  know  indeed,  that  among  the  laity  of  all  classes  of  society 
(naturally  also  in  romance  literature),  to-day  still,  the  conceptions  "hysteri- 
cal" and  "man-crazy"  are  nearly  synonymous.  No  wonder  then,  that  even 
from  the  chain  of  thought  of  physicians,  the  teachings  of  Hippocrates  only 
disappeared  in  the  course  of  the  eighteenth  century.  For  these  the  equally 
incorrect  view  that  hysteria  and  hypochondria  (this  last  term  we  must  to-day 
replace  by  "neurasthenia")  were  the  same  disease  was  substituted;  only  the 
first  was  the  special  privilege  of  the  female,  the  last,  that  of  the  male  sex.  It 
is  not  the  least  service  of  Charcot  that  he  made  clear  that  hysteria  and  neuras- 
thenia are  different  conditions  and  that  each  may  affect  both  sexes.  As  to 
hysteria  indeed,  its  great  preponderance  in  the  female  sex  was  recognized  by 
Charcot,  and  statistics  of  later  authors  give  this  as  85  to  90  per  cent.,  but 
the  determining  factor  for  this  predisposition  of  women  is,  as  our  later  dis- 
cussion of  the  nature  and  causes  of  hysteria  will  show,  not  the  female  genital 
organs,  but  the  female  psyche,  in  which,  in  comparison  to  the  male,  a  prepon- 
derance of  phantasy  and  conceptional  life,  as  well  as  diminution  in  the  power 
of  judgment  and  of  critical  inspection,  are  evident  Also,  men  who  become 
hysterical  are  throughout  of  the  so-called  "feminine  natures"   (which,  as  is 

436 


THE    PSYCHOXEUROSES  437 

understood,  is  meant  only  in  the  psychic  sense  and  may  be  combined  with  the 
most  virile  physical  make-up).  In  analogous  manner  the  predisposition  in 
childhood  as  well  as  that  of  certain  peoples  (South  Europeans,  Slavs,  Jews) 
is  to  be  explained. 

Only  as  a  curiosity  does  the  theory  of  W.  A.  Freund  introduced  not  so 
long  ago,  according  to  which  a  causal  connection  between  hysterical  phe- 
nomena and  gjmecological  diseases — for  example,  contraction  of  the  broad  liga- 
ments— exists  (in  the  sense  of  a  reflex  neurosis)  need  mention.  This  develop- 
ment of  a  one-sided  specialistic  view  has  unfortunately  led  to  the  removal  of 
the  pelvic  organs  in  hysterical  women,  by  which  the  psychoneurosis  was  natu- 
rally not  extirpated.  To-day,  fortunately,  the  operative  era  in  the  treatment 
of  hysteria  can  be  considered  as  definitely  closed. 

Symptomatology 

As  we  did  in  the  case  of  neurasthenia,  in  hysteria  we  will  first  describe  the 
different  disease  manifestations,  mainly  in  their  most  important  clinical  fea- 
tures, only  after  this  to  enter  upon  the  more  difficult  task  of  an  etiological 
pathogenetic  consideration.  The  symptomatological  description  of  hysteria 
is  made  easier  for  us  from  the  fact  that  a  po'-tion  of  the  symptoms  is  common 
to  both  psychoneuroses ;  this  concerns  namely,  such  phenomena  in  which  ex- 
aggerated self-observation  plays  a  mediating  role  as  we  have  sketched  it  in 
Lecture  XXVII.  Still,  the  intensity  of  these  disturbances,  corresponding  to 
the  incomparably  greater  auto-suggestibility  of  hysterics,  is  usually  much 
more  marked;  and  further  from  their  increased  auto-suggestibility  there  are 
also  qualitative  differences:  since  only  upon  a  basis  of  hysteria,  never  upon 
that  of  neurasthenia,  can  paralyses,  contractures  and  anesthesias  (of  sensible 
or  sensory  nature)  occur.  Special  diagnostic  importance  is  finally  to  be  at- 
tached to  the  "hysterical  character''  and  to  the  special  psychic  disturbances 
of  hysterics.  These  anomalies  manifest,  however,  such  intimate  connections) 
with  the  nature  of  the  disease  that  we  will  first  study  them  along  with  the 
pathogenetic  relations.  To  bring  some  order  into  the  striking  multiplicity  of 
the  hysterical  phenomena,  it  is  advisable  to  subject  the  permanent  symptoms, 
(stigmata)  the  hysterical  attacks,  paralyses  and  contractures,  to  a  separate 
description. 

1.     PERMANEXT  SYMPTOMS  ("HYSTERICAL  STIGMATA") 

a.     Sensible  axi>  Sknsoky  Symptoms 

The  majority  of  hysterics  complain  of  pain  of  one  sort  or  another.  In- 
dividually very  variable  in  localization,  they  usually  differentiate  themselves 
from  pains  as  they  occur  in  "hyper-algesic  neurasthenia"  through  their  greater 
intensity,  or  let  us  say  rather,  through  the  greater  activity  of  the  expressions 
of  pain  (complaining,  crying,  etc.).  Particularly  frequent  are  hysterica]  pains 
in  the  hack,  also  hysterical  headaches,  which  last,  as  a  rule,  are  not  described 
as  pressure  in  the  head  only,  but  as  a  throbbing,  burning  or  boring  pain,  which 
is   felt   by   the  majority  of  patients  superficially,   that    is,  in   the  scalp,  and   not 


438 


LECTURE    XXIX 


inside  the  skull.  Usually  there  is  with  this,  marked  hyperesthesia  of  the  hairy 
region,  pressure  or  even  touching  the  scalp,  a  slight  pulling  on  the  hair,  pro- 
duce lively  expressions  of  pain ;  percussion  is  described  as  "actual  torture." 
Very  frequently  headache  and  hyperesthesia  center  themselves  in  circumscribed 
regions  over  the  top  of  the  skull  (see  Fig.  110)  ;  the  simile  of  a  nail  driven  into 
one  of  these  areas,  occasionally  used  by  hysterical  patients,  has  led  to  the 
denomination  of  this  symptom  "Clavus  hystericus."  There  is  also  a  hysterical 
pain  in  the  face  which  is  differentiated  from 
neuralgic  prosopalgia,  above  everything  by  the 
continuity  and  diffuse  nature  of  the  pain  and 
in  contradistinction  to  that,  is  very  often  bi- 
lateral. The  greatest  intensity  of  pain  is  usu- 
ally located  in  the  temporal  region ;  in  many 
cases  the  pain  occurs  day  after  day  at  some 
definite  time — habit  pains  (Brissaud).  Ana- 
logues of  the  clavus  hystericus,  that  is  cir- 
cumscribed sensitiveness  to  pressure,  often  also 
spontaneously  painful  areas,  the  so-called 
"topalgias,"  occur  on  certain  portions  of  the 
trunk  (Fig.  110)  with  a  certain  predilection. 
By  the  Charcot  school  so  much  industry  was 
expended  upon  the  study  of  these  "mammary," 
"sternal,"  "ovarian"  points,  etc.,  that  one  can- 
not escape  the  impression  that  just  this  search- 
ing for  hyperesthesia  over  these  "classical" 
areas,  then  as  to-day,  frequently  unintention- 
ally suggested  to  the  patient  the  phenomena 
sought.  Nevertheless,  just  the  possibility  of 
conjuring  up  in  this  fashion  hyperesthesias  and 
pains,  is  a  clinically  very  useful  symptom  of 
hysteria;  further,  the  examination  of  healthy 
persons  shows  that  the  apex  of  the  skull,  as 
well  as  the  other  areas  of  predilection  for  topal- 
gias often  are  somewhat  more  sensitive  to  pres- 
sure than  the  rest  of  the  body  surface,  so  that 
perhaps  the  physiologically  caused  local  pre- 
disposition may  also  come  into  play.  That  the 
so-called  "ovarian  point"  has  nothing  to  do  with  the  ovaries  is  shown  by  the 
fact  that  this  symptom  also  occurs  in  male  hysterics.  Since  occasionally  by 
the  production  of  pain  on  pressure  over  the  spots  of  circumscribed  hyperes- 
thesia, hysterical  attacks  may  be  set  up,  on  the  other  hand,  however,  those 
already  in  progress  may  be  cut  short;  such  areas  have  been  denominated 
"hysterogenic"  and  "hysterofrenatory"  zones. 

Diagnostic  difficulties  are  presented  sometimes  by  hysterical  arthralgias, 
as  they  may  localize  themselves  in  joints  to  which  the  attention  of  the  pa- 
tient has  been  directed  by  some  sort  of  material  anomaly,  for  instance,  genu 


Typical  Localizations  of  Hysterical 
Topalgias  (Shaded)  and  Anes- 
thesias   (Black). 


THE    PSYCHONEUROSES  439 

valgum,  crackling  of  the  joint  surfaces,  etc.  Often,  indeed,  the  surgical  or 
roentgenological  findings  are  entirely  normal,  or  the  hysterical  nature  of  the 
pains  is  clear  from  their  localization  at  an  anatomically  indifferent  point  of 
the  joint  region.  For  the  rest,  however,  the  diagnosis  can  only  be  made  after 
long  observation.  Of  important  indication  is  particularly  the  disappearance 
of  joint  pains  when  the  attention  is  distracted — which  also  applies  to  the 
other  hysterical  "psychalgias."  These  last  are,  however,  not  always  of  such 
circumscribed  nature  as  in  the  examples  already  mentioned.  They  can  affect 
the  whole  body  (pantalgia),  or  more  rarely,  one  entire  half  of  the  body 
(hemialgia).  When  the  patient  on  account  of  hysterical  pains  which  occur 
or  are  increased  by  every  movement,  is  condemned  to  the  most  absolute  help- 
lessness, we  speak  with  Mobius  of  "Akinesia  algera";  where  every  touch  of 
the  skin  calls  forth  pain,  with  Pitres,  of  "Haphalgcsia." 

Of  greater  diagnostic  importance  than  pains  and  hyperesthesias  are  the 
hypesthesias  and  anesthesias  of  the  hysterical.  Indeed,  as  already  emphasized, 
just  through  the  occurrence  of  such  disturbances  which  are  foreign  to  neuras- 
thenia, hysteria  assumes  a  special  clinical  position  in  the  circle  of  psychoneurotic 
conditions. 

What  I  remarked  when  speaking  of  hysterogenic  zones,  about  the  sug- 
gestive production  of  hysterical  symptoms  by  the  physician  applies  here  also 
and  there  is  no  doubt  that  hysterical  anesthesias  can  be  provoked  by  the 
examiner  in  the  patient  intentionally  or  not,  that  further,  by  repeated  examina- 
tions (which  hence  are  to  be  avoided  as  far  as  possible  as  soon  as  tin-  hysteri- 
cal nature  of  the  disease  is  certain)  they  usually  increase  in  intensity  and 
extent.  On  this  account  never  test  the  sensibility  in  a  patient  suspected  to  be 
hysterical  in  the  way  of  asking  him,  "Do  you  feel  the  prick  equally  plainly 
upon  both  sides?"  or  "Is  there  any  place  where  you  do  not  feel  the  prick?" 
This  is  as  if  you  had  in  mind  directing  his  suggestibility  toward  the  test.  If 
you  wish  to  rule  this  out  as  far  as  possible  I  would  advise  you  to  avoid  all 
questioning  during  the  examination,  since  the  patient  (not  before  examined) 
usually  spontaneously  remarks:  "In  this  location  I  have  not  felt  the  prick," 
or  when  upon  application  of  a  strong  faradic  current  to  the  right  hand  he  ex- 
presses  pain,  to  the  left,  however,  hi'  remains  passive^  then  you  know  at  least" 
that  you  have  not  exercised  any  foreign  suggestion  and  only  autosuggestion 
remains  in  question. 

That  suggestion  in  genera]  often  plays  the  most  important  role  in  the 
production  of  hysterical  anesthesias  is  proved  by  the  fact  that  such  disturb- 
ances of  sensibility  can  be  cured  by  suggestion,  and  indeed,  may  be  changed 
topographically  thereby  (which  is  called  transference).  Why,  however,  the 
suggestibility  of  Hie  hysteric  manifests  it-elf  in  circumscribed  loss,  or  reduc 
lion  of  sensation  (and  besides  this  Iii  quite  characteristic  distribution)  remains 
a  puzzle.  While  the  generally  diffused  conception  of  motor  paralysis  permits 
us,  in  almost  every  hysterically  paralyzed  patient,  the  presumption  that  he 
has  already  seen  paralyzed  persons,  or  at  least  has  heard  of  them,  with  regard 
to  the  anesthesias,  similar  relations  are  not  to  be  assumed,  hence  observations 
of  suddenly  appearing  hysterica]   hemianesthesias  as   have  been   published   by 


440  LECTURE    XXIX 

Stierlin,  myself  and  other  authors,  in  previously  healthy  individuals,  as  a  result 
of  severe  fright  (railroad  accidents,  earthquakes,  etc.),  compel  us  to  assume 
that  some  organic  regularities  in  the  apparatus  of  apperception  supply  a 
ready-made  pattern  through  which  the  psychogenic  disturbance  receives  its 
clinical  stamp. 

The  hysterical  disturbances  of  sensibility  vary  greatly  in  intensity  from 
case  to  case  and  there  are  all  possible  transitions  from  slight  hypesthesia  to 
complete  anesthesia.  Still,  in  contradistinction  to  the  anesthesias  caused  by 
organic  nervous  diseases,  the  paradoxical  fact  is  noticeable  that  the  movements 
of  extremities  which  upon  testing  sensibility  show  themselves  totally  insensible, 
occur  in  entirely  normal,  coordinated  and  undisturbed  fashion,  that  with  an 
anesthetic  hand  the  patient  can  write,  play  the  piano,  etc.  Also  the  tendon 
reflexes  of  the  region  are  usually  not  diminished,  indeed,  they  are  often  in- 
creased as  in  neurasthenia,  only  the  mucous  membrane  reflexes- — pharyngeal 
reflex,  conjunctival  reflex — are  in  hysterics  relatively  often  very  weak,  or  in- 
deed, absent.  Sometimes  upon  pinching  or  pricking  the  anesthetic  cheek,  dila- 
tation of  the  pupil  occurs.  Sometimes  the  different  qualities  of  sensation  are 
affected  to  a  different  degree,  only  pain  sense  may  be  noticeably  disturbed. 
An  important  characteristic  of  the  hysterical  disturbances  of  sensibility,  their 
suggestive  influenceability  ("Pithiatism"  according  to  Babinski)  naturally  can 
only  be  tested  with  considerable  reserve.  Attempts  at  "transference"  for 
example,  in  which  through  any  hocus-pocus,  an  anesthesia  is  transferred  from 
one  side  to  the  other,  should  be  directly  forbidden,  since  they  distinctly  in- 
crease the  morbid  suggestibility.  As  to  the  topography  of  hysterical  disturb- 
ances of  sensibility,  we  can  distinguish  1,  universal  anesthesia  (excessively 
rare)  ;  2,  hemianesthesia ;  3,  circular  or  geometrical  anesthesias ;  4,  insular 
or  disseminated  anesthesias.  Typical  for  hysterical  hemianesthesia,  which 
usually  affects  also  the  mucous  membranes,  is  in  contradistinction  to  the  or- 
ganic form,  its  sharp  limitation  to  the  middle  line.  Also,  circular  anesthesias 
are  separated  from  the  normally  feeling  skin  areas,  by  very  sharp  lines  which 
usually  run  at  right  angles  to  the  long  axis  of  the  extremity — so-called  "ampu- 
tation lines"  (see  Fig.  110);  we  find  instead  of  peripheral  or  radicular  areas, 
cap-like,  sleeve-like,  sock-like,  glove-like,  finger-like,  anesthesias.  Of  organic 
diseases  only  lepra  sometimes  gives  similar  pictures.  That  between  the  dif- 
ferent topographical  types  of  hysterical  anesthesia  there  are  transitions  I 
need  not  specially  emphasize.  Experience  teaches  that  hysterical  anesthesias 
more  frequently  occur  upon  the  left  side.  Perhaps  this  coincides  with  that,  as 
van  Bieri'liet  has  shown,  in  healthy  people  there  is  usually  a  slight  difference 
in  the  sensibility  of  the  skin  unfavorable  to  the  left  side,  when  tested  by  delicate 
methods. 

Very  frequent  is  the  combination  of  hysterical  disturbances  of  general 
sensibility,  with  those  of  the  sense  organs  in  which  there  are  naturally  phe- 
nomena analogous  in  principal.  Among  these  stands  first  in  importance,  con- 
centric limitation  of  the  visual  field,  in  confirming  which  with  the  perimeter, 
we  notice  in  many  cases  that  the  visual  field,  in  the  course  of  the  investigation, 
becomes  constantly  smaller;  also  that  the  mutual  relations  of  the  color  fields 
have  experienced  a  change  from  the  normal.     Namely,  in  healthy  people,  the 


THE    PSYCHOXEUROSES  441 

visual  field  for  green  is  smallest,  somewhat  larger  is  that  for  red,  still  larger  the 
area  for  yellow,  particularly,  however,  that  for  blue.  In  hysterical  dvschroma- 
topsia  (we  speak  of  Forster's  type  of  alteration)  on  the  other  hand,  the  limits 
for  red  more  or  less  overlap  those  for  blue.  In  spite  of  the  most  pronounced 
hysterical  narrowing  0f  the  visual  field  the  patients  can  usually  move  about  with 
entire  safety  without  striking  against  anything  and  have  no  suspicion  of  the 
anomaly  appearing  upon  perimetric  examination.  Occasionally,  however,  there 
is  a  hysterical  amblyopia  proper  in  which,  besides  the  visual  field,  acuteness  of 
vision,  color  sense  and  light  sense  are  found  diminished.  Hysterical  blindness 
is,  on  the  other  hand,  quite  rare;  like  hysterical  amblyopia,  it  occurs  with  in- 
tact light  reflex  of  the  pupils.  A  very  great  rarity  is  monocular  polyopia.  The 
hysterical  disturbances  of  vision  occur,  as  a  rule,  upon  both  sides,  still,  they 
are  generally  most  marked  upon  the  side  on  which  general  sensibility  is  most 
affected.  Xext  to  vision,  taste  is  most  frequently  affected  in  hysteria ;  now 
it  is  perversions  of  taste  in  which  bitter  or  otherwise  disagreeable  tasting  sub- 
stances are  found  agreeable,  and  eaten  by  preference  (hysterical  '"longings," 
'"pica")  again,  ageusia  for  single  or  for  all  qualities  of  taste.  This  ageusia 
can  occur  uni-  or  bi-laterally.  Less  frequent  is  hysterical  anosmia,  indiffer- 
ently unilateral  or  bilateral,  still  rarer  hysterical  dullness  of  hearing  which, 
as  a  rule,  occurring  unilaterally,  can  sometimes  increase  to  complete  deafness. 
The  different  tests  for  hearing  (see  Lecture  II)  give  the  same  results  as  in 
nerve  deafness  ('"Rhine"),  positive,  etc.,  but  the  subjective  ear  noises,  so  fre- 
quent in  organic  diseases  of  the  auditory  nerve  apparatus,  are  almost  always 
absent;  further,  the  patient,  deaf  on  one  side,  usually  in  conversation  conducts 
himself  like  a  healthy  person,  while  in  unilateral  organic  hypacusis  of  any  con- 
siderable degree,  we  nearly  always  notice  that  the  patient  attempts  to  turn  his 
better  ear  toward  us.  Sometimes  hysterical  deafness  is  an  elective  one.  in  that, 
for  example,  whispers  are  heard  while  there  is  complete  deafness  for  the  tick- 
ing of  a  watch,  etc. 

b.     Vasomotor  Symptoms 

If  we  disregard  the  not  infrequent  combination  of  hysteria  with  different 
vasomotor  neuroses  (see  Lecture  XXV),  as  well  as  the  disturbances  of  vas- 
cular innervation  frequent  also  in  neurasthenia  (dermographism,*  factitious 
urticaria,  fleeting  erythema,  "dead  finger,"  etc.),  the  vasomotor  anomalies  oc- 
curring in  the  region  of  hysterical  anesthesias  are  especially  to  be  mentioned. 

The  absence  of  bleeding  on  deep  stabbing  with  a  needle  has  a  peculiar  im- 
portance in   the  history  of  civilization.     The   famous  "Malleus  maleficarum" 

was  accounted  one  of  tin-  surest  "witch  signs"  and  it  has  hence  contributed  to 
the  delivery  of  innumerable  victims  to  the  slake 

Sometimes  there  appears  also  about   the  stall  a  circumscribed  oedema, 

Further,  in  hysteria  there  is  a  not  entirely  rare,  firm  swelling,  not  pitting 

*  In  hysterica  soim-limcs  more  complicated  forms  of  dermographism  occur:  for  example, 
in  one  ut  my  patients,  upon  stroking  tin-  Bkin  with  tin-  finger,  there  appeared  one  red  be- 
tween two  white  streaks. 


442  LECTURE    XXIX 

upon  pressure,  combined  with  intense  cyanosis  and  coldness  of  the  skin  over 
the  anesthetic  or  paralyzed  hand  which  has  been  called  "blue  oedema."  The 
circumstance  that  in  persons  especially  susceptible  to  hypnosis  it  has  been 
possible  to  suggest  the  appearance  of  skin  vesicles  and  sugillations,  suggest 
the  possibility  that  trophic  skin  disturbances  and  extravasations  of  blood  may 
occur  upon  the  basis  of  hysteria.  Still,  it  is  certain  that  the  impressive  ma- 
jority (if  not  indeed  all)  the  cases  described  as  "hysterical  pemphigus," 
"hysterical  ulceration,"  "hysterical  hemorrhages,"  etc.,  have  been  produced 
by  self-injury,  a  point  to  which,  when  speaking  of  the  hysterical  psyche,  we 
will  come  back  again.  Also  most  cases  of  the  so-called  "hysterical  fever"  are 
to  be  referred  to  simulation  (warming  or  shaking  the  thermometer),  further, 
diagnostic  errors — failure  to  recognize  a  latent  tuberculous  focus,  etc. — often 
occur.  On  the  other  hand  there  are  known  a  number  of  undoubted  cases,  in 
which  the  temperatures  were  taken  by  the  physician  himself  in  the  rectum  or 
vagina  and  the  eventuality  of  an  organic  cause  could  be  excluded.  These  are 
chiefly  of  temperature  disturbances  which  occurred  in  hysterical  seizures  or  at 
the  time  of  the  menses,  some  reaching  quite  high  degrees  (up  to  42°  C.=  106° 
F.),  but  were  not  accompanied  either  by  corresponding  alterations  of  pulse 
and  respiration,  or  by  the  urinary  changes  characteristic  for  fever.  On  this 
account  the  name  "hysterical  hyperthermia"  is  decidedly  to  be  preferred. 
Palpitation  of  the  heart  and  rapidity  of  the  pulse  occur  in  hysteria  much  less 
frequently  than  in  neurasthenia. 

c.     Motor  Symptoms 

Hysterical  tremor  can  be  unilateral  or  bilateral,  and  may  affect  the  upper 
or  the  lower  limbs.  In  Basle  as  the  "shaking  disease"  it  excited  great  wonder 
some  3'ears  ago :  A  hysterica]  school-girl  had  in  this  way  exerted  so  striking 
an  effect  upon  the  phantasy  of  her  school-mates  that  the  tremor  gradually 
spread  from  child  to  child.  In  this  manner  two  hysterical  school  epidemics 
occurred,  which  were  only  stamped  out  by  strictly  enforced  isolation.  For  the 
rest,  we  observe  hysterical  tremor  with  special  frequency  among  "traumatic 
hysterics."  It  is  usually  in  a  great  measure  dependent  upon  attention,  much 
more  so  than  is  the  case  in  the  tremor  of  neurasthenics  or  even  in  tremor  organi- 
cally caused.  Only  in  a  minority  of  cases  is  it  the  fine  vibrating  tremor  men- 
tioned in  Lecture  XXVII,  usually  it  is  of  greater  amplitude  and  moderate 
rapidity  (5  to  7  oscillations  a  second)  :  Its  character  can,  however,  be  subject 
to  frequent  change  ("polymorphous  tremor").  Further,  occasionally  the 
most  varied  dyskinesias  can  be  "imitated"  by  hysteria,  in  which  long  observa- 
tion, careful  taking  of  the  history  and  accurate  study  of  the  suggestive 
influenceability  is  needed  to  determine  the  hysterical  nature  of  the  disease. 
Here  belong  among  other  things  the  disease  pictures  which  we  have  already 
mentioned  in  Lecture  V  as  "Chorea  Major"  and  "Bergerons'  Electric 
Chorea." 

The  frequent  coincidence  of  hysteria  and  local  spasms  as  well  as  tics  has 
been  alluded  to  when  speaking  of  the  dyskinesias;  I  would  mention  particularly, 


THE    PSYCHONEUROSES  443 

oesophagism,  pharyngism,*  yawning,  sneezing,  snoring  spasms,  facial  tic,  etc. 
(see  pages  67  and  72). 

Anesthetic  limbs  upon  being  tested  with  the  dynamometer  usually  show  a 
diminution  of  the  gross  strength  with  very  rapid  fatigability,  more  rarely,  what 
has  been  described  by  Charcot  as  the  "contracture  diathesis."  Massage, 
faradization  or  binding  the  affected  extremity  has  as  its  result  a  contracture 
which  only  ceases  again  after  some  seconds  or  minutes. 

J.     A  iscerai>  Disturbances 

On  the  part  of  the  digestive  organs  practically  the  most  important,  since  in 
severe  cases  they  lead  to  marked  inanition  and  cachexia,  are  hysterica]  anorexia 
and  hysterical  vomiting.  In  the  last,  the  food  is  usually  vomited  immediately 
after  taking;  also  independent  of  taking  food,  there  is  bringing  up  of  masses 
of  mucus.  Very  often  in  hysteria,  in  contradistinction  to  neurasthenia,  vom- 
iting is  the  only  anomaly  on  the  part  of  the  stomach  and  the  feeling  of  pres- 
sure in  the  epigastrium,  eructations  and  the  other  symptoms  of  nervous 
dyspepsia  are  absent.  That  only  an  exact  examination  of  the  stomach  with 
exclusion  of  an  organic  affection  permits  us  to  diagnose  the  vomiting  as  hysteri- 
cal is  self-evident.  Aery  typical  of  hysteria  are  the  cases  in  which  patients 
regularly  react  with  vomiting  to  some  definite  situation  {e.g.,  coitus),  upon 
seeing  certain  colors,  hearing  certain  noises,  etc.,  or  where  they  are  able  to 
vomit  "at  command."  Meteorism,  the  distention  of  the  intestines  by  gas, 
reaches  on  an  average  much  higher  degrees  than  in  neurasthenics;  in  hysterics, 
as  a  rule,  the  swallowing  of  air  (aerophagia)  plaj's  a  part  in  this.  As  curiosi- 
ties nia\  hi'  mentioned  here  the  cases  of  "hysterical  pregnancy,"  in  which  women 
not  pregnant  but  filled  with  longing  for  a  child,  have  shown  along  with  a 
meteoristic  distention  of  the  abdomen,  cessation  of  the  menses,  swelling  of  the 
breasts,  the  appearance  of  colostrum,  morning  sickness,  etc.,  in  short,  the  most 
varied  signs  of  pregnancy,  and  only  upon  the  supposed  beginning  of  labor, 
was  the  actual  nature  of  the  case  maele  evident.  Obstinate  constipation  is 
particularly  frequent  in  hysterics;  also  the  mucous  colic  already  mentioned  is 
often  a  symptom  of  hysteria.  On  the  part  of  the  uropoetic  apparatus  the 
mosl  importanl  hysterica]  anomalies  arc  oliguria  which  usually  (to  a  certain 
extent  vicariously) ,  ,H"e>  hand  in  hand  with  hysterica]  VOmiting,f  and  polyuria 
(up  to  12  liters  a  day  and  more)  ;  dm-  to  polydipsia.  Particularly  frequent  are 
genital  disturbances;  almost  all  female  hysterics  suffer  from  nervous  symp- 
toms  at  menstruation;  sexual  frigidity  i--  frequent,  with  which  are  contrasted 
an  exalted  psychic  eroticism  and  a  resulting  "desire  for  excitement."  On  the 
other  hand  sexual  hyperesthesia  occurs  frequently.  It  leads  often  to  a  vaginis- 
mus hindering  cohabitation,  or  the  introitus  directly  represents  a  hysterogenic 

Globus  hystericus,  with  which  we  will  become  acquainted  as  .-in  initial  symptom  of 
hysterical  convulsive  attacks,  which,  however,  <><<nrs  in  man)  patients  without  relation  I" 
Midi  attacks,  i--  probably  a  variety  of  pharyngism  speciallj  colored  by  accompanying 
paresthesias. 

fin  the  vomited  matter,  even  in  cases  in  which  ii  could  nol  In'  assumed  thai  Hie  patient 
had  swallowed  urine,  urea  has  been   frequentlj    recognized. 


444  LECTURE    XXIX 

zone.  Many  hysterical  men,  in  spite  of  erotic  monomania,  are  impotent  or  of 
feeble  potence.  This  explains  in  part  the  sexual  aberrations  in  such  individuals, 
while  in  other  perverse  hysterics  the  "psychopathia  sexualis"  (homosexuality, 
sadism,  fetishism,  etc.)  must  be  considered  as  a  primary  psychic  stigma  of 
degeneration. 

2.     HYSTERICAL    SEIZURES 

When  in  the  70's  and  80's  of  the  last  century  through  the  brilliant  de- 
scriptions of  Charcot  and  his  pupil  Richet,  the  interest  of  the  whole  medical 
world  in  hysteria  was  aroused  anew,  for  a  long  time  the  study  of  the  so-called 
attacks  of  "grand  hysteria"  occupied  everywhere  the  foreground.  To  this 
circumstance  it  is  proper  to  attribute  the  fact  that  at  that  period  these  "classi- 
cal" paroxysms  were  much  more  frequently  observed  than  now.  At  that  time 
the  great  role  which  suggestion,  imitation  and  other  psychogenic  factors  play 
in  the  disease  picture  of  hysteria,  in  the  great  enthusiasm  for  the  work  of 
symptomatological  detail,  was  entirely  overlooked,  so  that  patients  with 
hysterical  attacks  were  placed  in  a  common  ward  with  other  psychoneurotics, 
were  demonstrated  clinically  as  "show  cases,"  and  the  physicians  discussed  in 
the  presence  of  their  patients  with  imprudent  naivete  the  picture  of  these 
paroxysms,  which  besides  this,  through  newspaper  and  literary  elaborations, 
though  distorted,  reached  a  more  extended  audience.  To-day,  however,  such 
cases  when  they  occur  are  isolated  as  far  as  possible;  in  the  interest  of  the 
patient  we  avoid  clinical  demonstration,  and  otherwise  withhold  all  concern  in 
the  attack  which  may  act  suggestively.  So  it  has  come  about  that  the  "grand 
hysterical  attack,"  as  Charcot  and  Richet  described  it,  now  occurs  quite  rarely ; 
while  in  Charcot's  time,  at  the  Salpetriere,  day  by  day  such  paroxysms  oc- 
curred in  different  patients.  During  my  stay  at  this  giant  hospital  in  190-1- 
1905,  I  was  not  able  to  study  one  single  case  of  major  hysteria,  and  still,  if 
I  may  so  express  it,  they  had  "learned"  their  attacks  under  Charcot. 

When  now  I  lay  before  you  the  theatrical  picture  of  this  most  peculiar 
manifestation  of  hysteria,  I  would  not  have  the  differentiation  of  the  four 
stages  of  the  "grand  attack"  understood  in  the  schematic  sense  which  Charcot's 
school  gave  to  it.  The  symptomatology  of  the  attack,  indeed,  is  subject  to 
considerable  variation  from  patient  to  patient ;  nevertheless,  there  come  under 
observation  from  time  to  time  cases  which  with  some  good  will  can  be  de- 
nominated typical  and  regular.  In  such  a  case  there  appear  at  first  certain 
disturbances,  which  in  analogy  with  the  epileptic  paroxysms,  may  be  called  the 
"hysterical  aura":  particularly  typical  is  the  feeling  of  a  ball  arising  from 
the  abdomen  or  from  the  epigastrium  up  into  the  throat  ("globus  hystericus"), 
combined  with  great  anxiety,  palpitation  of  the  heart,  tinnitus  aurium,  dizzi- 
ness, darkening  of  the  visual  field,  etc.  Then  begins  the  first  stage  of  the 
attack  proper,  the  epileptiform,  characterized  by  general  tonic  and  clonic  con- 
vulsions. As  a  difference  from  the  true  epileptic  attacks,  the  patients  do  not 
usually  fall  down,  but  sink  down  and  do  not  suffer  any  injuries;  also  the 
piercing  initial  scream  of  the  epileptic  is  wanting  and  the  face  does  not  be- 
come cyanotic.  After  some  time  the  epileptiform  stage  passes  over  into  that 
of  "contortions  and  grand  movements"  or  of  "clownism";  the  patient  makes 


THE    PSYCHOXEUROSES  445 

faces,  throws  the  arms  about,  throws  herself  around;  like  the  "bridge"  cus- 
tomary in  wrestling,  the  body  rests  only  upon  the  occiput  and  the  heels  ("arc 
de  cercle"),  flings  her  legs  about,  stamps,  rolls  about  her  long  axis,  produces 
by  tension  of  the  neck  muscles  the  "hysterical  swollen  neck"  ("Blahals"), 
makes  movements  with  the  pelvis,  etc.  There  follows  now  the  period  of  "pas- 
sionate attitudes"  in  which,  apparently  under  the  influence  of  dream-like  altera- 
tions of  consciousness,  the  patient  mimics  certain  emotions,  for  example,  fear, 
erotic  rapture,  religious  ecstasy,  deep  grief,  etc.  Sometimes  these  conditions 
find  expression  simultaneously  also  by  screaming,  moaning,  declarations  of 
love,  lascivious  grunting,  singing,  praying,  etc.  Without  sharp  delimitation 
this  phase  of  the  grand  attack  passes  over  into  the  "terminal  delirium,"  in 
which  the  mimicry  ceases  and  there  is  chiefly  vocal  expression  of  hallucinatory 
perceptions  {e.g.,  visions  of  animals).  A  "major"  hysterical  seizure  lasts  on 
the  average  from  a  quarter  to  half  an  hour.  After  the  attack,  the  patient 
sometimes  remains  for  a  time  in  a  cataleptic  condition,  usually,  however,  she1 
immediately  gets  up,  and  in  spite  of  the  great  motor  efforts  which  she  has 
made,  shows  no  fatigue  worth  mentioning.  Sometimes,  however,  the  attack 
leaves  behind  hysterical  paralyses  or  contractures,  later  to  be  described. 

Much  more  frequent  than  the  major  attacks,  are  the  so-called  minor 
attacks,  whose  manifestations  may  be  quite  various.  Usually  they  are  epilep- 
tiform, more  rarely  onlyr  the  phase  of  contortions  is  assumed  ("demoniacal 
attacks").  Other  seizures  occur  only  as  loss  of  consciousness  with  slight  mus- 
cular tension  ("syncopal  attacks"),  or  a  sudden  falling  asleep  ("lethargic 
attacks"),  or  as  catalepsy,  in  which  the  patient  with  consciousness  fully  re- 
tained, passes  into  a  condition  of  complete  immobility.  Xot  at  all  infrequently 
we  observe  the  so-called  "affect  and  respiratory  spasms,"  that  is,  laughing 
spasm,  weeping  spasm,  screaming  spasm,  tachynoic  attacks  (in  which  the  fre- 
quency of  respiration  may  increase  to  180  per  minute — figures  which  in  general 
only  occur  in  hysteria,  and  which  hence  may  be  considered  as  pathognomonic)  ; 
further,  attacks  of  "hysterical  barking  cough,"  particularly  unpleasant  for  the 
surrounders.  As  "psychic  equivalents"  of  the  hysterical  convulsive  attack, 
may  be  mentioned  attacks  of  sleep-walking  (somnambulism),  Ganser's  symp- 
tom ("symptom  of  approximate  answers" ).  that  is,  incorrect  answers  to  the 
simplest  questions,  although  the  patient  is  apparently  clear  mentally,*  "ecmne- 
sia,"  by  which  expression  is  meant  psychic  paroxysms  in  which  the  patients 
reproduce  events  of  their  childhood  in  word  and  gesture. 

In  the  epileptiform  attacks  under  certain  conditions  a  differentiation  from 
the  morbus  sacer  may  be  quite  difficult,  usually,  however,  on  the  basis  of  the 
criteria  which  I  have  collected  in  the  following  table,  the  differential  diagnosis 
can  be  made  without  great  difficulty. 

'Characteristic  of  Ganser's  symptom  is  the  fad  thai  while  Ihe  answers  of  Ihe  patient 
seem  incorreei  and  Inappropriate,  they  nevertheless  have  a  certain  connection  with  the  mat- 
ter in  hand. — {Translator.) 


446 


LECTURE    XXIX 


Etiology 

Sensory   aura 
Initial  cry 
Fall 

Biting  the  tongue 
Color  of  the  face 


Often  after  some  definite  causa- 
tion (rage,  fright,  etc.,  provo- 
cable   by   suggestion) 

Usually  absent 

Usually   absent 

Gradual  sinking  down;  the  pa- 
tient almost  never  suffers  injury 

Very  rare 

Usually  not  markedly  altered 


Usually  without  apparent  causa- 
tion; never  provocable  by  sug- 
gestion 

Often   present 

Often  present 

Sudden  falling;   injuries  frequent 

Very  frequent 

Initial  paling,  then  marked  cyano- 


Discharge  of  urine 

Pupils 

Consciousness 


Duration 
Terminal  sleep 


Possibility  of  being 
cut  short 


Almost  never 

Only  in  very  rare  cases  reaction- 
less 

Not  entirely  lost;  the  patient  re- 
acts to  tickling  the  mucous 
membrane  of  the  nose,  sprink- 
ling with   cold  water,  etc. 

Often  one  half  hour  to  an  hour 


Almost  always  absent,  sometimes 
sudden  coming  to 

Frequently  can  be  (hystero-frc- 
natory  zones,  suggestive  influ- 
ence, external   irritants,  etc.) 


Very  frequent 
Dilated   and   rigid 


Apart      from      status     epilepticus, 
only  minutes 

Present 
Absent 


3.     HYSTERICAL    PARALYSES 

Hysterical  paralyses  usually  appear  suddenly  either  in  connection  with  an 
attack  of  hysterical  convulsions,  or  independent  of  this,  in  consequence  of  an 
emotion — fright,  anxiety,  rage,  grief,  etc.  Also  the  physical  traumata  with 
which  hysterical  paralyses  are  relatively  frequently  connected,  act  only  through 
the  medium  of  the  affect  accompanying  them,  but  have  on  the  other  hand  in 
so  far  pathogenetic  importance,  since  they  usually  determine  the  localization 
of  the  paralyses,  in  that  this  affects  the  part  exposed  to  violence.  The  trauma 
can  be  very  slight,  even  purely  imaginary:  so,  in  a  case  of  my  observation  a 
blow  directed  at  the  patient  was  caught  upon  his  open  umbrella  and  did  not 
touch  the  paralyzed  arm  at  all.  After  explanation  to  the  patient  of  the  un- 
reality of  the  supposed  trauma,  the  severe  paralysis  rapidly  recovered. 

Sometimes  after  the  trauma  a  certain  time  elapses  before  the  paralysis 
appears:  in  such  cases  the  conception  that  a  paralysis  may  result  gains  the 
upper  hand  only  after  the  accident  and  leads  by  autosuggestion  to  the  elimina- 
tion of  the  innervations  in  question.  This  method  in  which  the  patient  "hatches 
out"  his  paralysis  is  not  at  all  infrequent  in  "accident  hysteria"  (which  also 
may  manifest  itself  by  anesthesia,  spasms,  contractures,  etc.).  The  disease 
producing  action  of  definite  nosological  conceptional  complexes  is  here  favored 
by  the  factors  which  we  have  already  considered  under  accident  neurasthenia 


THE    PSYCHONEUROSES  U7 

(page  i'24>,  etc.).  We  hence  speak  also  of  "Pension  hysteria."  Hysterical 
paralyses  are  usually  flaccid,  more  rarely  spastic,  and  belong  iij\v  to  the  mono- 
plegic,  again  to  the  heniiplegic  or  paraplegic  type.  They  may  represent  transi- 
tory, fleeting  phenomena  (this  applies  particularly  to  post-paroxysmal  paraly- 
ses which  Gendrin  hence  considers  as  exhaustion  paralyses),  on  the  other  hand, 
however,  are  marked  by  great  obstinacy.  The  combination  with  hypesthesias 
or  anesthesias  of  corresponding  topography  is  very  frequent.  Hysterical 
paralyses  when  they  persist  very  long  can  lead  to  a  definite  reduction  in  size 
of  the  affected  limbs,  still,  this  is  only  an  atrophy  from  disuse  which  is  never 
accompanied  by  reaction  of  degeneration  or  change  of  the  superficial  outlines. 

The  reflexes  are  never  lost  in  the  flaccid  form;  in  the  spastic  form  they 
are  usually  somewhat  exaggerated,  but  not  greatly  so.  The  phenomena  of 
Babinski,  Oppenheim  and  Mendel-Bechterew  are  always  absent  as  is  also  true 
ankle  clonus,  while  we  occasionally  find  the  "pseudo-ankle  clonus"  already  de- 
scribed. Oppenheim  has  further  pointed  out  that  the  examiner  who  holds  a 
hysterically  paralyzed  extremity  for  some  time  in  his  hand  sometimes  plainly 
perceives  occasional  innervation  impulses  in  its  muscles  and  that  such  a  limb, 
when  passively  held  in  a  certain  position,  then  suddenly  let  loose,  is  able  to 
maintain  for  some  time  a  position,  which  is  only  explainable  by  the  activity  of 
the  otherwise  paralyzed  muscles.  Hysterical  hemiplegia  almost  always  avoids 
the  facial  and  hypo-glossus.  The  gait  differs  markedly  from  that  of  cerebral 
hemiplegia,  in  that  the  paralyzed  leg  is  not  circumducted,  but  is  simply  dragged 
along,  the  sole  or  the  heel  scraping  the  ground.  If  the  patient  uses  a  crutch, 
he  particularly  does  not  bring  his  leg  into  contact  with  the  floor.  In  hysterical 
paraplegias  the  functions  of  bladder  and  rectum  are  almost  always  intact;  in 
all  hysterical  paralyses  tropho-vasoniotor  disturbances  are  much  rarer  and  in 
every  case  less  intense  than  in  the  organic.  Finally,  it  is  pathogenetically  as 
will  as  diagnostically  important  that  only  the  conscious  voluntary  movements 
are  suspended.  In  alcoholic  and  chloroform  intoxications,  on  the  contrary, 
there  is  lively  gesticulation. 

A  very  characteristic  syndrome  is  the  so-called  "astasia-abasia,"  in  which 
the  patienl  in  bed  can  execute  every  movement  with  normal  strength  ami  com 
pleteness,  but,  on  the  contrary,  upon  attempting  to  walk  or  to  stand,  simply 
sinks  down  (different  from  this  symptom-complex  first  described  by  Jaccoud, 
is  Mingazzini's  "Stasobasophobia,"  an  emotionally  caused  psychasthenic  dis- 
turbance of  gait  marly  related  lo  agoraphobia). 

A  frequent  form  of  hysterical  paralysis  is  hysterical  aphonia,  usually  the 
immediate  effeel  of  a  fright,  a  particularly  obstinate  symptom,  tending  to  re- 
cur. The  patients  speak  only  in  whispers;  in  it.  as  laryngoscopic  examination 
Bhows,  the  vocal  chords  are  not  at  all,  or  only  imperfectly  approximated. 
Coughing  and  clearing  the  throat  usually  occur  noisily,  however.  Rare,  but 
almost  pathognomonic  of  hysteria  is  suddenly  appearing  mutism,  in  which,  in 
contradistinction  to  aphasia,  no  sound  or  portion  of  a  word  can  be  produced, 
while  mimic  movements  remain  quite  active.  Excessively  rare  is  hysterical 
ptosis,  which,  according  to  Oppenheim,  is  to  be  distinguished  from  thai  organ- 
ically caused,  by  the  fact  thai  the  contraction  of  the  frontal  muscle  observed 
in  this  last   i^  absent;  il  should  no!  be  confused  with  hysterical  pscudo  ptosis 


448  LECTURE    XXIX 

which  occurs   from  spasmodic  closure  of  the  eyes,  also  introduces  us  to  the 
hysterical  contractures. 

4.     HYSTERICAL  CONTRACTURES 

In  these  contractures  there  is  permanent  fixation  of  a  limb  in  a  definite 
position  by  the  tonic  contraction  of  certain  muscles.  They  develop  in  contra- 
distinction to  the  hysterical  paralyses  by  degrees  in  the  majority  of  cases, 
though  usually  in  connection  with  the  exciting  causes  mentioned  under  those 
paralyses.  Upon  distraction  of  the  attention,  the  contracture  diminishes  (in 
sleep  it  usually  disappears),  while,  on  the  contrary,  the  manipulation  by  the 
examiner  of  the  contractured  limb  plainly  increases  the  muscular  tension.  In 
very  long  duration  of  the  hysterical  contracture  there  appear,  however,  as 
in  mechanically  caused  fixation  in  a  definite  position,  material  alterations  in 
the  muscles,  the  ligaments,  etc.,  which  are  responsible  for  the  fact  that  some- 
mes  even  upon  cure  of  the  psychogenic  anomaly  a  complete  restitutio  ad 
integrum  is  not  attainable.  In  contradistinction  to  the  contracture  after 
organic  diseases  of  the  pyramidal  tracts,  in  hysterical  contractures  (they  also 
assume  by  preference  in  the  arm,  the  type  of  "flexion,"  in  the  leg  that  of  "ex- 
tension"), the  reflexes  are  not  exaggerated  and  the  other  accompanying  phe- 
nomena of  the  spastic  symptom-complex  are  absent.  Also  upon  passive  short- 
ening of  the  distance  between  the  origin  and  the  insertion  of  the  contracted 
muscles  a  relaxation  does  not  occur.  Along  with  the  contractures  of  the  ex- 
tremities and  the  pscudo-ptosis  already  mentioned  as  relatively  frequent  in 
hysteria,  there  are  to  be  named  contractures  of  the  neck  muscles  (see  in  Lec- 
ture IV,  under  Tonic  Local  Spasms,  Torticollis,  Retrocollis,  etc.)  and  glosso- 
labial  hemispasm  which  draws  the  lips  and  tongue  laterally,  in  a  grotesque 
fashion. 

Psychology  and  Pathogenesis 

Gentlemen  !  Many  physicians,  when  asked  their  position  upon  the  ques- 
tion as  to  the  nature  of  this  peculiar  psychoneurosis  whose  semiological  study 
we  have  now  finished,  confess  still  to-day  the  standpoint  of  Lasegue,  "The 
definition  of  hysteria  has  never  been  given  and  never  will  be."  This  convenient 
axiom  we  can  only  accept  in  the  sense  that  it  is  not  possible  to  include  the 
varied  symptomatology  of  this  "protean  neurosis,"  the  munifoldness  of  its 
special  etiological  factors  and  the  numerous  variations  in  its  course,  within  a 
short  formula.  It  is,  however,  possible  to  arrive  at  a  pathogenetic  conception 
of  what  authenticates  itself  as  hysterical  if  one  attempts  to  view  the  tangle  of 
clinical  details  from  a  psychological  point  of  view. 

If  we  start  out  with  the  description  of  the  psychic  stigmata  of  hysteria, 
whose  discussion  we  have  intentionally  put  off  until  this  time,  we  can  distinguish 
with  Cramer  four  groups  of  phenomena  which  naturally,  like  the  somatic  stig- 
mata, need  not  all  be  present  in  every  individual  case,  of  which,  however,  one 
or  more  occur  in  definite  or  discrete  manner  in  most  hysterics.  These  are:  1, 
Excitability  under  affect;  2,  ideas  of  being  wronged;  3,  inability  to  accurately 
reproduce  impressions  ;  4,   striking  variations  of  mood.      In   general  the  hys- 


THE    PSYCHONEUROSES  449 

fcerica]  individual  is  moved  with  extraordinary  intensity  by  affects  of  agreeable 
or  disagreeable  tone,  and  reacts  to  these  emotions  in  a  specially  unrestrained 
manner.  In  general  a  morbid  egocentrism  manifests  itself  in  the  tendency  to 
interpret  occurrences  in  their  relation  to  his  own  person  and  in  so  doing  bj 
preference  to  assume  the  role  of  a  martyr;  in  him  in  general  the  retouching  of 
the  objective  facts  through  subjective  factors,  as  well  as  the  description  of 
external  happenings,  particularly  in  that  of  his  own  sufferings,  reaches. the 
highest  degree,  even  "pathological  untruthfulness  ;*'  finally,  in  general,  the 
hysterical  are  entirely  irresponsible  in  their  disposition. 

It  is  a  question  now  if  this  "hysterical  character"  furnishes  a  comprehensive 
and  satisfactory  explanation  for  the  fact  that  just  in  such  individuals  auto- 
suggestibility  (whose  importance  for  the  occurrence  of  hysterical  phenomena 
can  be  experimentally  recognized)  is  increased  to  an  actual  disease  condition. 
This  question  is  to  be  answered  in  the  negative  upon  the  ground  that  many 
healthy  people,  but  particularly  many  psychasthenics  and  neurasthenics,  pre- 
sent one  or  another  of  these  stigmata,  or  indeed,  all  of  them,  in  marked  devel- 
opment, and  even  then,  do  not  show  specific  hysterical  symptoms  if  they  experi- 
ence severe  psychic  traumata. 

Further,  Dubois  has  pointed  out  in  a  brilliant  manner,  that  autosugges- 
tibility,  in  conjunction  with  the  constitutional  inferiorities  expressing  them- 
selves in  the  hysterical  character,  may,  indeed,  explain  the  appearance  of 
inadequate  somatic  reactions  to  emotions,  not,  however,  the  following  impor- 
tant criteria:  "The  persistence  during  weeks,  months,  years,  indeed  during  I  he 
whole  life,  of  an  array  of  functional  disturbances  which  appear  in  the  absence 
of  every  primary  material  injury  and  which  in  consequence  must  he  referred 
to  psychic  causes."  This  characteristic  fixation  of  the  somatic  phenomena 
arising  from  emotions  (actual  or  suggested),  Dubois  attributes  to  a  psychic 
anomaly  which  is  to  be  considered  the  most  important  factor  in  the  specific 
hysterical  diathesis,  "sense  impressionability." 

Now  the  Bern  neurologist  defines  "sense  impressionability,"  "The  ability  to 
impress  upon  the  sensations  arising  from  affects,  the  stamp  of  reality:"  you 
see  in  how  satisfactory  an  agreement  what  I  remarked  to  you  about  the  role  of 
phantasy  as  a  predisposing  factor  for  hysterical  affects,  stands  with  the  dedue 
tions  of  Dubois.  That,  also,  in  the  stigma  the  inability  to  accurately  repro 
duce  impressions— which  can  increase  to  "mythomania,"  "phantasy,"  reaches 
clinical  expression,  has  scarcely  escaped  you. 

Though  I  confess  myself  without  reserve  an  adherenl  <>.  the  teachings  of 
Dubois  as  to  the  pathogenesis  of  hysterical  conditions,  I  cannot  leave  unmen- 
tioned  the  much-discussed  views  of  the  Vienna  neurologist  Freud.  He  assumes 
that  an  affect  when  it  does  not  had  to  an  adequate  reaction,  is  transposed  into 
somatic  disturbances.  The  hysterical  disease  manifestations  are  based  upon 
such  a  "conversion."  And,  indeed,  the  disease-producing  "complex"  is  regU 
larlv  to  he  sought  in  the  sexual  experiences  of  the  earliest  childhood,  which 
had  exercised  a  powerful  impression  upon  the  childish  psyche,  hut  from  natural 
reasons  i-  concealed  and  not  "reacted  out."  In  that,  tittle  by  little  this  com 
plex  disappears  from  consciousness,  that  is.  i-  "supplanted"  bj  hysterical 
phenomena  (which,  all  the  -ana.  ar>'  Bymptomatologically  in  accord  »ilh  the- 


450  LECTURE    XXIX 

sexual  trauma  in  childhood,  for  example,  a  hysterical  attack  with  a  parental 
coitus  witnessed),  from  a  therapeutic  point  of  view  it  is  necessary  through 
"psycho-analysis"  to  bring  up  the  "pent  in  affect"  from  the  subconsciousness 
into  consciousness  and  rid  the  patient  of  it. 

That  in  spite  of  the  very  great  exaggeration  of  the  frequency  of  an 
infantile  sexual  etiology,  the  extensive  lack  of  criticism  in  the  development 
of  the  doctrine  of  conversion,  and  the  regrettable  growth  which  the  thera- 
peutic application  of  psychoanalysis  has  attained,  Freud's  views  contain  a 
useful  principle,  is  to  be  acknowledged  without  dispute.  So  he  has  pointed 
out  effectively,  not  indeed  for  the  first  time,  but  most  clearly,  that  every 
hysteria  is  a  traumatic  one,  and  that  it  is  regularly  psychic  factors  which 
bring  the  dormant  psychoneurosis  to  an  outbreak.  These  are  mainly  acute 
and  brutal  psychic  traumata,  and  not,  as  a  rule  (as  is  generally  the  case  in 
acquired  neurasthenia)  a  more  chronic  emotional  unrest,  the  storm  of  affective 
irritations,  small  in  themselves,  but  always  protracted  in  action,  which  sup- 
porting themselves  upon  former  ones  not  yet  run  out,  undergo  summation 
and  accumulation.  It  is  also  true,  that  not  rarely  the  symptomatology  of 
the  concrete  hysterical  attack  is  influenced  by  the  initial  trauma.  I  have 
already  mentioned  this  for  accident  hysteria.  As  to  sexual  traumatic  hysteria, 
a  like  relationship  is  sometimes  found,  so  three  cases  of  contracture  of  the 
adductors  of  the  thigh  which  I  observed  in  females  arose  from  attempts  at 
criminal  assault,  on  which  occasion  these  muscles  were  put  into  a  position 
to  do  honor  to  their  ancient  name  "custodes  virginitatis."  Let  it  be  re- 
marked in  passing,  no  "psycho-analysis"  is  needed  to  uncover  sexual  etiological' 
factors  (or  to  suggest  them)  ;  they  are  naturally  not  subconscious  and  "dis- 
placed," but  usually  they  are  intentionally  concealed  from  the  physician  until 
he  has  inspired  a  sufficient  confidence  by  tactful  demeanor.  That  then  talking 
it  over  with  the  psychotherapeutist  and  his  soothing  reassurance  can  only 
act  advantageously  and  can  assist  recovery,  is  evident  to  any  one  without  his 
having  to  confess  adherence  to  the  dogma  of  the  "hemming  in  of  affects." 
The  Psalmist  wrote  about  "sin,"  "Should  I  conceal  it,  my  limbs  would  give 
way,"  and  also  the  ordinance  of  auricular  confession  bespeaks  eminent  psycho- 
logical intuition.  Still  one  thing  more :  it  is  shown  that  many  cases  of 
hysteria  can  be  dated  back  to  initial  traumata  of  sexual  or  erotic  nature; 
this  corresponds  to  the  powerful  role  which  the  sexual  factor  plays  in  the 
life  of  the  individual.  Only  one  must  not  think  that  the  sexual  traumata 
must  always  occur  in  early  childhood,  and  must  be  connected  with  actual 
irritation  of  the  genitals,  as  Freud  has  apodictically  affirmed.  Rather  do 
sentimental-erotic  misfortunes,  as,  for  example,  a  broken  engagement  in  a 
young  girl,  play  a  very  great  role.  Also  in  the  frequent  hysteria  of  childless 
women,  the  insufficient  potence  of  the  husband  is  but  rarely  to  be  held  respon- 
sible;  much  more  frequent  is  the  collapse  of  a  long-cherished  hope  of  offspring; 
the  same  thing  applies  to  the  hysteria  of  old  maids,  which  often  breaks  out 
at  the  time  when  the  last  illusion  in  regard  to  the  longed-for  "settlement" 
is  dissipated. 

Dubois  has  said  of  the  female  hysteric,  that  she  is  a  comedienne,  but  does 
not  know   it,   that   she   is    acting,   and  honestly   believes   in   the  truth   of   her 


THE    PSYCHONEUROSES 


451 


roles.  This  may  in  general  be  entirely  correct,  but  does  not  apply  to  certain 
cases  in  which  the  desire  for  commiseration,  the  "pose."  the  coquetting  with 
illness,  lead  to  refined  "pathomimicry"  which  sometimes  does  not  draw  back 
from  self-mutilation.  Fig.  Ill  represents  the  hand  of  an  hysterical  woman 
who  was  detected  as  she  was  producing  her  "hysterical  pemphigus"  with  hot 
sealing  wax.  Still  more  monstrous  was  the  case  of  a  young  girl  who  had 
produced  in  herself  a  "puzzling  tropho- 
neurosis" by  about  150  cuts  reaching 
down  to  the  fascia  on  the  left  arm 
and  hot Ii  legs  and  in  whom  my  diag- 
nosis of  self-injury  nearly  brought  me 
into  an  unpleasant  situation  for  sus- 
pecting the  "brave  daughter"  of  such  a 
tiling.  Fortunately  the  discovery  of  a 
pair  of  bloody  scissors  concealed  be- 
tween the  mattresses  rehabilitated  me, 
and  the  patient  then  furnished  an  inter- 
esting written  account,  from  which  it 
appeared  that  after  an  exciting  family 
scene,  while  in  her  bath,  she  accidentally 
noticed  the  anesthesia  of  her  left  arm, 
and  then,  partly  in  order  to  make  her- 
self interesting  and  partly  to  mystify 
the  family  doctor,  had  begun  the  auto- 
mutilation.  In  another  girl,  when  at 
different  points  in  her  skin  needles  ap- 
peared, I  received  the  confession  that 
she  had  swallowed  them  in  order  to  pro- 
voke a  hemorrhage  from  the  stomach. 
In  a  case  of  Dietilafoy's  the  patient 
allowed  it  to  go  so  far  that  the  ex- 
tremities which  he  had  intentionally 
cauterized  with  lye  were  amputated. 
Opposed  to  these  rare  extremes,  forc- 
ing up  the  thermometer,  the  production 
of  blood-spitting  by  wounding  the 
gums,  putting  albumen  in  the  urine,  etc., 
represent  the  frequent  "formes  frustes"  Hysterlcaj  Self-Injur 
of  pathomimicry.  From  the  fact  that 
self-injury,  swallowing  foreign  bodies,  etc.,  occasionally  result  fatally,  tin- 
otherwise  good  prognosis  of  hysteria  as  regards  life  becomes  more  serious  in 
such  cases.  The  same  remark  applies  to  the  sometimes  theatrically  staged 
suicidal  attempts,  which  occasionally   succeed  better  than  was  intended.      True, 

intentionally  carried  out  suicide  is  exceedingly  rare  in  hysterics. 

But   one   word   on    the   so-called    "hysterical    psychoses."       I    am   not    willing 

to  accept  without  reserve  the  view  of  Aschaffenburg,  according  t<>  which  there 
is  no  definite  clinical   form  of  hysterical  insanity,  bul    rather  that   usually  a 


Fig.  in. 


Pseudo  Pemphigus. 


452  LECTURE    XXIX 

mixture  of  more  or  less   numerous   and  striking  hysterical   features  with   the 
most  varied  psychoses  (for  example,  with  manic  depressive  insanity)  can  occur. 

Treatment 

That  from  what  has  been  said  above,  the  rational  treatment  of  hysteria 
can  only  be  a  psychic  one  is  apparent  without  further  remark.  While  here, 
in  general,  the  same  underlying  principles  apply  as  were  laid  down  for  the 
psychic  treatment  of  neurasthenia,  in  the  previous  lecture,  on  account  of  the 
preponderant  role  which  in  the  clinical  picture  of  hysteria  is  to  be  assigned 
to  autosuggestive  factors,  some  modifications  are  desirable. 

While,  for  example,  the  neurasthenic  is  favorably  influenced  psychically 
by  frequent  and  accurate  examination  into  his  condition,  since  he  thereby 
becomes  assured  that  the  physician  is  carrying  out  his  task  particularly 
thoroughly,  and  on  this  account  is  inclined  to  put  more  and  more  faith  in 
the  assurance  that  there  is  no  organic  disease,  and  his  troubles  are  bagatelles, 
in  hysterics  it  is  well  to  avoid  frequent  examinations.  For  example,  if  by  an 
exact  examination  carried  out  under  all  precautions,  an  anesthesia  is  recog- 
nized as  certainly  hysterical,  it  is  wisest  to  completely  ignore  it  thereafter 
until  taking  the  condition  of  the  patient  upon  discharge.  In  severe  cases  of 
hysteria,  isolation  from  the  home  surroundings  and  bed  treatment,  not  ambula- 
tory, is  absolutely  required.  In  hysterics  much  reduced  in  nutrition  this  can 
be  combined  with  the  rest  cure  after  Weir  Mitchell.  Anorexic  hysterics  must 
have  the  psychical  genesis  of  their  repugnance  to  taking  food  explained  to  them 
and  after  this  must,  without  severity  but  with  unyielding  persistence,  be 
trained  to  take  an  increasing  quantity  of  nourishment.  One  should  refrain  as 
far  as  possible  from  using  the  oesophageal  tube.  While  a  few  patients  have  a 
wholesome  horror  of  this  instrument  and  so  may  be  persuaded  to  take  a  suffi- 
cient quantity  of  food,  on  the  other  hand  the  patient  finds  pleasure  in  his  role 
of  martyr,  and  there  is  difficult}'  in  laying  the  ghost  that  has  been  called  up. 
Also  astasia-abasia,  aphonia,  contractures,  etc.,  must  be  cured  by  irresistible 
reassurance  and  patient  explanation  of  their  psychogenic  nature  by  methodical 
re-education  of  the  will.  Seizures  are  disregarded  as  far  as  possible;  also  the 
family  and  surrounders  must  learn  to  not  make  much  of  these,  but  to  content 
themselves,  after  sprinkling  the  patient  with  some  cold  water,  to  leave  him  alone 
as  much  as  possible  until  he  comes  to  himself.  In  paralyses,  aphonia,  etc.,  in 
fresh  still  untreated  cases,  the  so-called  "surprise  method"  is  often  found  a 
success.  In  this,  by  the  application  of  some  striking  procedure  previously  un- 
known to  the  patient  (the  application  of  static  electricity,  endo-laryngeal  fara- 
dization, hypnosis,  etc.),  a  sudden  cure  of  the  symptom  (naturally  not  of  the 
disease)  bordering  on  the  marvelous  may  be  sometimes  obtained.  Against  this 
method  no  objection  can  be  made,  only  if  we  explain  afterward  to  the  patient  the 
psychotherapeutic  nature  of  the  cure,  that  the  procedure  is  only  active  sug- 
gestively, so  proving  to  him  directly  the  autosuggestive  nature  of  his  disease. 
For  the  rest,  the  object  of  our  treatment  must  be  not  only  the  explanation  to 
the  patient  of  the  nature  of  his  troubles,  but  also  of  their  cause;  he  must 
learn  to  combat  his  "sense  impressionability,"  his  phantasy,  his  egocentrism, 


THE    PSYCHOXEUKOSES  453 

his  impulsiveness,  his  exaggerated  emotivity,  as  well  as  to  oppose  the  psychic 
traumata  which  has  set  up  his  hysteria,  with  objectivity  and  stoicism,  after 
he  liberates  himself  from  the  pressure  of  painful  memories  by  their  communi- 
cation to  a  sympathetic  counselor.  All  these  things  allow  themselves  to  be 
put  into  words  in  a  very  condensed  and  plausible  manner,  but  in  practice  are 
the  most  difficult  and  trying  tasks  which  come  to  the  psychotherapeutist. 


LECTURE  XXX 
Migraine 

In  the  course  of  these  lectures  we  have  become  acquainted  with  different 
varieties  of  headache  as  a  symptom  occurring  in  a  large  number  of  nervous 
diseases:  so  in  arteriosclerosis  and  syphilis  of  the  brain,  in  internal  hemor- 
rhagic pachymeningitis,  in  the  different  leptomeningitides,  in  brain  tumors, 
brain  abscesses,  hydrocephalus,  in  progressive  paralysis,  after  epileptic  at- 
tacks, hysteria,  etc.  The  occurrence  of  headache  (cephalalgia,  cephak-ea)  ex- 
tends, however,  far  beyond  the  boundaries  of  neurology.  I  would  remind  you 
of  those  forms  which  are  known  to  you  as  an  accompaniment  of  fevers,  as  the 
expression  of  slight  uremia,  in  consequence  of  chronic  nephritis,  as  an  initial 
symptom  of  syphilitic  infection;  further,  of  the  locally  caused  headache  in 
diseases  of  the  ear,  the  eye,  the  frontal  sinus  and  antrum  of  Highmore,  of  the 
bones  of  the  skull  (gummata,  tumors,  caries,  periostitis),  of  the  neck  and 
frontal  muscles,  and  the  cephahea  of  chronic  lead,  alcohol,  nicotine  and  many 
other  intoxications,  of  those  which  are  complained  of  during  the  incubation 
stage  of  infectious  diseases,  of  the  transitory  headache  with  which  even  the 
healthy  person  becomes  acquainted,  after  mental  or  physical  overexertion, 
excesses  in  drinking,  sleepless  nights,  sojourn  in  hot  and  poorly  ventilated 
rooms,  etc.  To-day,  however,  there  remains  for  us  the  description  of  a  form 
of  headache  which  even  in  the  second  century  after  Christ  was  described  as  a 
disease  sui  generis  (by  Galen  and  Aretwus,  of  Cappadocia),  and  still  pre- 
serves its  nosological  autonomy.  This  is  hemicrania  or  migraine.  Just  as  in 
Lecture  XXVI,  we  kept  in  mind  only  genuine,  not  symptomatic  epilepsy,  here 
also  we  will  disregard  those  cases  in  which  migraine-like  attacks  occur  during 
the  development  of  a  brain  tumor,  as  symptoms  of  multiple  sclerosis,  or  pro- 
gressive paralysis;  also  those  observations  very  interesting  in  themselves,  where 
in  epileptics  migraine  attacks  alternated  with  convulsive  seizures,  so  that  their 
conception  as  "epileptic  equivalents"  has  impressed  itself.  Such  cases,  like  the 
not  very  rare  occurrence  of  members  of  one  and  the  same  family,  afflicted  part 
with  epilepsy  and  part  with  hemicrania,  suggests  the  thought  of  a  certain 
nosological  relationship  between  the  two  affections  (to  which  besides  the  oc- 
currence in  paroxysms  is  common),  while  on  the  other  hand,  opposed  to  such 
a  conception,  is  the  fact  that  epilepsy  usually  manifests  progressive  tendencies ; 
migraine,  on  the  contrary,  shows  practically  always  with  the  advance  of  age, 
a  reduction  in  the  frequency  and  intensity  of  the  attacks  and  indeed,  in  many 
cases  finally  spontaneously  disappears.  Also  in  migraine  patients  degenerative 
symptoms  are,  as  a  rule,  absent. 

It  is  an  exceedingly  frequent  nervous  disease,  particularly  among  the  upper 

454 


MIGRAINE  453 

classes  of  societ}-,  which  affects  women  in  a  quite  preponderant  manner;  in 
my  material  at  the  most  20  per  cent,  of  the  hemicranics  were  of  the  male  sex. 
Like  heredity,  it  is  often  recognizable.  That  the  arthritic  diathesis,  which  is 
much  supported  by  the  French  and  English,  plays  any  role  worth  speaking  of  in 
the  etiology  of  migraine,  is  very  questionable;  in  any  case,  the  notorious  rarity 
of  true  gout  in  our  neighborhood  stands  in  striking  contrast  to  the  extent  of 
migraine.  The  combination  with  psychoses,  neurasthenia  and  hysteria  is  quite 
frequent;  that  with  vasomotor  neuroses  not  rare.  Very  questionable  is  the 
frequently  affirmed  causal  relationship  of  migraine  to  gynecological,  rhino- 
logical,  or  stomach  diseases. 

The  disease  appears  usually  at  the  time  of  puberty,  still,  its  beginning  at 
an  even  earlier  age,  for  example,  0  ax  7  years,  is  in  no  way  rare.  Quite  90 
per  cent,  of  cases  set  in  before  the  20th  3'ear  of  life;  an  occurrence  of  at- 
tacks of  migraine  for  the  first  time  in  persons  between  40  and  50  years  of  age 
is  always  suggestive  of  the  symptomatic  form.  Relatively  frequently  the  at- 
tacks cease  during  the  6th  decade,  after  it  has  decreased  in  severity  and  fre- 
quency from  the  40th  year;  in  women  the  recovery  sometimes  coincides  exactly 
with  the  menopause.  On  the  other  hand,  there  are  persons  who  are  still  sub- 
ject to  migraine  attacks  even  at  an  advanced  age. 

Sy  mp  t  o  m  a  tology 

In  the  preponderant  majority  of  hemicranics — if  we  disregard  the  ac- 
companying psychoneurotic  conditions — the  migraine  attacks  occurring  at 
more  or  less  regular  intervals  (it  can  show  a  regular  or  an  irregular  type), 
represent  the  whole  clinical  picture  and  in  the  intervals  there  are  no  anomalies 
to  be  observed.  A  minority  of  patients,  on  the  other  hand,  show  also  inter- 
paroxysmal  symptoms.  We  will  now,  to  gain  a  comprehensive  view,  firs! 
sketch  the  usual  clinical  picture  of  migraine  attacks,  then  consider  the  differenl 
varieties  of  the  paroxysms,  and  finally  the  interparoxysmal  phenomena, 

a.      The  Regular  Migraine  Attack,  Hemicrania  Simplex 

For  the  occurrence  of  a  migraine  attack,  there  are  either  no  provocative 
agents  to  be  found,  or  certain  exciting  causes  can  witli  more  or  less  proba- 
bility be  held  responsible;  this  most  certainly  applies  to  the  menstruation, 
since  many  patients  are  exclusively  and  regularly  affected  by  hemicrania  at 
the  time  of  their  catamenia;  for  the  rest,  Hie  patients  frequently  give  as  ex- 
citing factors  overexertion,  psychical  excitement,  coitus,  excessive  use  of  al- 
cohol, too  short  or  too  long  sleep,  indigestion,  hunger,  the  occurrence  of  sultry 
weather,  etc.  The  time  of  predilection  for  the  outbreak  of  the  attack  is  the 
early  forenoon,  still  it  can  occur  at  any  hour.  Occasionally  it  strikes  the  pa- 
tient like  lightning  out  of  a  clear  sky,  though  he  has  gone  lo  lied  with  a  clear 
head  and  feeling  particularly  well,  lie  wakes  in  the  morning  with  severe  mi- 
graine; usual] v,  however,  characteristic  premonitory  symptoms  precede  its  ap 
pearance.  Grasset,  Rauzier,  and  others  have  differentiated  two  types  of  these 
prodromi,  the  "excited  type"  in  which  there  conns  oxer  the  patient  a  peculiar 


456  LECTURE    XXX 

motor  unrest  along  with  psychic  irritability,  and  sometimes  also  sexual  excite^ 
ment,  and  the  "depressed  type"  which  is  characterized  by  the  preponderance 
of  a  sad  or  morose  mood  with  a  feeling  of  depression,  by  drowsiness  and 
yawning.  When  the  attack  is  coming  on,  the  patients  usually  look  pale  and 
prostrated;  often  they  complain  of  pressure  in  the  epigastrium,  precordial 
pressure,  urinary  irritability,  shivering,  dullness  in  the  head ;  when  the  mi- 
graine comes  on  in  the  morning,  during  the  preceding  night,  sleep  is  often 
restless  and  accompanied  by  bad,  in  part  stereotyped,  dreams.  An  actual 
"aura"  occurs  only  in  a  few  severe  forms  of  migraine,  for  example,  in  the  form 
of  paresthesia  of  the  tongue,  or  of  one  hand,  tinnitus  aurium,  spots  before  the 
eyes,  etc. 

The  headache  is,  as  the  name  hemicrania  expresses,  at  the  start  one-sided 
or  predominating  upon  one  side,  but  not  at  all  infrequently  in  the  course  of  the 
attack  affects  both  sides  of  the  head  with  equal  intensity.  It  is  usually  frontal 
or  temporal,  more  rarely  occipital,  but  can  also  radiate  into  the  neck,  shoulder 
and  arm.  Its  severity  usually  increases  little  by  little  to  remain  at  its  maxi- 
mum several  hours,  and  then  just  as  gradually  to  disappear  again;  when  the 
migraine  has  so  far  decreased  that  the  patient  can  go  to  sleep  he  usually  awakes 
with  his  head  quite  free  again,  rarer  is  a  "critical"  discontinuance  of  the  pain 
while  the  patient  is  awake.  Its  severity  is  quite  different  from  case  to  case, 
sometimes  also  in  the  same  patient  from  attack  to  attack.  With  the  mildest 
migraine  the  patient  can  still  go  about  his  business,  the  more  severe  forms 
compel  him  to  lie  down  in  a  darkened  room  as  free  from  noise  as  possible 
(since  light  and  noise  increase  his  trouble)  ;  the  most  severe  cases,  however, 
increase  to  actual  torture  which  forces  from  the  patient  groans  and  lamenta- 
tions. The  character  of  the  pain  is  varied  ;  usually  it  is  described  as  hammer- 
ing or  boring,  sometimes  as  cutting  or  burning.  As  to  its  topography,  it  is 
by  no  means  always  stereotyped ;  even  in  the  unilateral  type,  sometimes  the 
right,  again  the  left,  now  more  the  forehead,  now  more  the  temporal  region 
can  be  affected,  etc. 

The  migraine  headache  is  always  accompanied  by  nausea,  which  usually  in- 
creases to  retching,  often  to  actual  vomiting;  this  last  is  independent  of  taking 
nourishment.  It  can  occur  upon  an  empty  stomach  (for  example,  immediately 
after  awakening),  with  the  bringing  up  of  gastric  juice  or  bile,  or  there  are 
indeed,  abortive  vomiting  movements,  a  particularly  painful  condition.  The 
loss  of  appetite  is  complete  during  the  attack. 

The  face  is  usually  pale  and  sunken  in  (the  so-called  "white"  or  "angio- 
spastic migraine"),  more  rarely  reddened  and  swollen  ("red"  or  "angio-para- 
lytic  migraine").  Sometimes  these  disturbances  of  vascular  innervation  are 
accompanied  also  by  other  sympathetic  phenomena ;  so  the  physiologist  Du 
Bois-Reymond  noticed  in  his  own  migraine  attacks,  besides  pallor  of  the  face, 
redness  and  heat  of  the  ear,  hyperemia  of  the  conjunctiva  and  narrowing  of 
the  palpebral  fissure.  Flatau  found,  at  the  height  of  the  hemicranic  paroxysm, 
hardening  of  the  temporal  arteries,  myosis  or  mydriasis,  swelling  of  the  upper 
lids,  etc.  Oppenheim  and  others  have  described  increased  discharge  of  mucus 
from  the  nose,  Tissot,  Labarraque  and  others,  small  extravasations  of  blood 
in  the  face,  in  the  nose,  in  the  retina.     Curschmann  noticed  "vasomotor  angina 


MIGRAINE  457 

pectoris."  Berger,  diarrhoeas,  Calmeil,  polyuria  and  pollakiuria,  I  myself  cir- 
cumscribed oedema  of  the  face,  "dead  fingers,"  and  flow  of  tears,  as  accom- 
paniment of  the  hemicranic  attacks.  The  pulse  is  sometimes  slowed,  indeed 
to  40  a  minute. 

Mobius  lias  pointed  out  the  relatively  frequent  occurrence  of  sensitiveness 
to  pressure  of  the  Vallcix's  points  of  the  trigeminus  and  occipitalis   (see   Lee 
hire  III,  pages  48-49.     Also  a  hyperesthesia  of  the  seal])  and  the  skin  of  the 
face  appears  during  the  migraine  attacks,  further,  paresthesias  of  the  lips,  the 
tongue  and  the  hand. 

The  attacks  just  sketched,  which  in  a  few  patients  show  themselves  only 
at  long  intervals  (about  two  or  three  times  in  the  year),  in  others  once  or 
twice  a  week,  upon  the  average,  however,  have  a  tendency  to  appear  about 
every  three  or  four  weeks,  can  in  severe  cases  succeed  one  another  in  such  a 
manner  that  there  is  a  status  hemicranicus  (or.  according  to  Oppenheim,  a 
"permanent  hemicrania").  In  this  after  the  migraine  is  apparently  on  the  de- 
crease, the  headache  increases  again  to  maximum  height,  and  this  repeats  itself 
for  several  days.  From  this  condition  Flat  ail  with  propriety  wishes  to  see 
separated  "continued  hemicrania."  that  is,  cases  in  which  the  migraine  instead 
of  the  average  duration  of  the  attack  of  about  12  hours,  persists  for  days  in 
unaltered  intensity,  and  then  slowly  disappears. 

b.      Particular  Varieties  of  Migraine 

Among  tin'  varieties  of  migraine,  on  account  of  their  frequency,  the  abortive 
or  rudimentary  attacks  deserve  to  be  mentioned  in  the  first  place;  in  these, 
after  more  or  less  definite  development  of  the  prodromal  symptoms,  there  is 
a.  slighl  pressure  in  the  head  with  some  nausea,  but  after  a  short  time  the 
patient    feels  entirely  well  again. 

Of  greater  symptomatic  interest  are  the  migraine  forms  which  occur  with 
cerebral  irritative  or  debet  symptoms;  so,  at  the  height  of  the  attack,  there 
are  not  so  very  rarely  more  or  less  outspoken  aphasic  disturbances  (usually 
of  Brora's,  more  ranly  of  Wernicke's  type);  further  FcrS  and  others  have 
pointed  out  fleeting  hemipareses  on  the  6pposite  side  to  the  maximum  of  pain; 
Liveing,  to  object  ive  disturbances  of  sensibility  of  cerebral  topography  :  Oppen- 
heim, to  transitory  typical  cerebellar  ataxia,  with  severe  vertigo  (cerebellar 
hemicrania);  Flatau,  to  clonic  contractions  on  one  side  of  the  face,  etc. 

A.s  "ophthalmic  migraine"  Charcot  and  Frrr  have  isolated  a  variety  of 
hemicrania,  in  whose  clinical  picture  certain  visual  disturbances  appear  par- 
ticularly prominently.  One  of  the  most  frequent  tonus  of  course  of  this  eye 
migraine,  is  the  following:  The  patient  has  suddenly  a  peculiar  light  per- 
ception, in  that  either  flames.  >parks  or  lightning,  move  before  his  eyes,  or 
with  darkening  of  the  central  part  of  his  visual  field,  in  its  periphery  bright 
serrations,  which  now  separate  from,  again  approach  one  another,  nou  rotate 
like  a  cog  w  In,!,  disappear,  again  glitter  with  all  colors  of  the  rainbow,  but 
do  nut  prevent  the  perception  of  surrounding  objects.  We  speak  of  "scintil 
lating  scotoma"  or  also  of  "teichopsia"  |  rampart)  since  the  serrated 

figures  remind  n>  of  the  plan  of  a  citadel  aft,  r  Vauban. 


458  LECTURE    XXX 

The  light  phenomena,  which  usually  occupy  only  one-half  of  the  visual 
field,  disappear  after  some  minutes,  usually  to  make  place  for  a  transitory 
hemianopsia,  eventually,  also,  for  a  temporary  amaurosis,  to  which  then,  the 
usually  specially  severe  unilateral  pain,  the  nausea,  the  prostration,  in  short, 
the  ordinary  migraine  symptoms,  succeed.  The  psychiatrist  Jolly  and  the 
astronomer  Airy  have  furnished  very  good  descriptions  and  pictures  of  their 
own  attacks  of  ophthalmic  migraine  and  scintillating  scotoma.  Ophthalmic 
migraine  can  also  begin  without  the  entoptic  phenomena  described,  with  simple 
temporary  hemianopsia  or  amaurosis  ;*  further,  the  ocular  symptoms  instead 
of  affecting  as  is  usual  both  eyes,  exceptionally  affect  only  one;  finally,  there 
is  sometimes  an  intense  sensitiveness  to  pressure  of  the  one  eye  ("iritic  mi- 
graine" of  Piorry).  Combination  of  ophthalmic  hemicrania  with  the  focal 
symptoms  already  enumerated  (transitory  aphasia,  facial  hemispasm,  etc.),  is 
relatively  frequent.  Antonelli  and  Siegrist  have  been  able  to  recognize  oph- 
thalmoscopically  during  the  attack,  a  spasm  of  the  retinal  vessels. 

As  ophthalmic  migraine  affects  the  visual  organ  itself,  ophthalmoplegic 
migraine  (denominated  by  Mobius  "periodic  oculomotor  paralysis")  involves 
its  muscles.  The  attack  begins  like  simple  hemicrania,  which,  however,  as  a 
rule,  is  characterized  by  great  severity  and  long  duration  (up  to  14  days)  ; 
then,  however,  it  passes  over  into  an  oculomotor  paralysis  (usually  total,  but 
irregularly  distributed  among  the  different  muscles).  This  last  is  homolateral 
with  the  headache  and  usually  lasts  for  several  weeks,  while  the  pain  generally 
disappears  with  the  appearance  of  the  ophthalmoplegia.  The  trochlearis  and 
abducens  are  only  rarely  affected. 

As  analogue  of  ophthalmoplegic  migraine,  Flatau  has  isolated  "facio- 
plegic"  (better,  "prosoplegic")  migraine,  a  form  unknown  to  me  personally, 
in  any  event  very  rare.  It  leaves  behind  a  facial  paralysis  of  peripheral  type 
which  recovers  only  after  several  weeks. 

We  will  close  the  enumeration  of  the  atypical  forms  of  migraine  with  the 
mention  of  "olfactory  migraine,"  "gustatory  migraine,"  and  "otic  migraine." 
These  are  attacks  which  are  accompanied  by  disturbances  of  smell,  taste  or 
hearing,  or  are  followed  by  such.  Such  observations  are  also  to  be  considered 
as  great  rarities. 

Entirely  outside  of  the  limits  of  hemicrania  are  to  be  placed  forms  of 
headache  which  have  been  described  by  Hartcnbcrg,  Peritz,  and  others,  as  "mi- 
graine of  the  arthritic,"  myalgic  migraine,  etc.  As  a  matter  of  fact,  these 
cases  belong  to  an  interesting  rheumatic  affection  which  has  been  isolated  by 
Hcnschcn,  Norstrom,  S.  Auerbach,  Edinger,  and  others,  as  "nodular"  or  "in- 
durative" headache.  In  this,  the  pain  radiates  from  the  neck  and  forehead 
muscles  unilaterally  or  bilaterally  over  the  scalp,  and  in  the  trapezius,  rhom- 
boideus,  splenii,  frontalis  and  other  muscles  there  are  to  be  recognized  upon 
palpation,  partly  diffuse,  partly  circumscribed  swellings.  Now  the  swelling  is 
yielding  and  elastic,  again  firm  and  hard.     The  first  corresponds  more  to  the 

*  One    of    ray    patients,    a    Polytechnic    student,    while    in    school,    noticed    the  onset    of 

hemianopsia,    in    that    suddenly,    instead    of   two   blackboards,   he   only    saw   that    to  the    left; 

immediately  afterward   bis  right  hand  and  right  side  of  his  tongue  "went  to  sleep,"  and  pain 
in  the  left  side  of  the  forehead  appeared. 


MIGRAINE  459 

subacute,  the  last  more  to  the  chronic  cases.  Almost  always  there  are  re- 
frigeratory influences,  sometimes  of  local  nature  (in  women  washing  the  hair 
in  winter)  to  be  discovered.  His  has  also  pointed  out  the  presence  of  uricemia. 
Under  the  influence  of  fatigue  and  dragging  upon  the  affected  muscles,  of 
local  refrigeration,  and  getting  wet,  this  nodular  or  rheumatic  cephalaea  usually 
exacerbates.  Besides  muscular  nodules  and  indurations,  enlarged  lymph 
glands,  and  infiltration  of  the  skin  at  the  back  of  the  neck  may  sometimes  be 
found.  Reflexly  (perhaps  also  by  irritation  of  the  sympathetic  in  the  neck), 
the  severity  of  the  pain  can  lead  to  nausea,  rarely  to  vomiting.  In  pieces 
removed  from  such  "headache  nodules"  I  have  not  been  able  to  find  anything 
abnormal  microscopically;  there  is  also  no  infiltration  or  true  induration,  but 
as  A.  Mutter  thinks,  the  result  of  a  localized  hypertonia.  Local  applications 
of  heat  combined  with  massage  and  percutaneous  or  internal  salicylic  treat- 
ment, is  if  persisted  in  long  enough,  of  sovereign  efficacy  in  this  "nodular 
headache." 

c.      Interparoxysmal  Symptoms 

Expressly  to  have  pointed  out  the  greatly  neglected  interparoxysmal 
symptoms  in  migraine  patients,  is  a  special  service  of  E.  Flatau.  Among  these 
are  on  the  one  hand  interesting,  those  syndromes  which  occur  themselves  in 
the  form  of  attacks  and  so  justify  their  consideration  as  "migraine  equiva- 
lents"; on  the  other  those  which  are  characterized  by  their  constancy.  Of 
migraine  equivalents  there  may  be  mentioned  as  examples:  Attacks  of  vaso- 
motor angina  pectoris,  Quincke's  oedema,  bronchial  asthma,  neuralgias  (for 
example,  Morton's  metatarsalgia),  rotary  vertigo,  tinnitus  aurium,  psychic 
depression,  gastralgia,  yawning  and  sneezing  spasms.  Of  the  permanent  symp- 
toms which  usually  affect  older  persons,  most  frequently  described  is  thCper- 
sistence  of  hemianopsias,  or  minor  defects  in  the  visual  field  after  repeated' 
occurrence  of  ophthalmic  migraine.  Meige  saw  develop  in  an  aged  migraine 
patient  a  hemiparesis  of  the  face  with  unilateral  oedema.  Exceptionally  oph- 
thalmoplegic migraine  can  leave  behind  permanent  paralysis  of  the  oculo- 
motorius  or  of  some  of  its  branches.  Finally,  I  would  mention  that  two  of 
inv  migraine  patients  had  permanent  bradycardia. 

Pathogenesis 

As  to  the  basis  of  migraine,  a  number  of  theories  have  been  proposed,  into 
all  of  which   I  naturally  cannot  go.     The  most   modern,  supported   by  S.   Auer- 

bach,  is  based  upon  Reichardt's  theory  of  "swelling  of  the  brain"  already 
touched  upon  in  Lecture  XIX.  Auerbach  thinks  that  the  symptom-complex 
migraine  is  explained  with  least  constraint,  if  we  assume  that  the  "hemicranic 
predisposition"  depends  upon  a  lack  of  relation  between  the  space  within  the 

skull  and   the  volume  of  the  brain,  and  that    the  attacks   are  set    up  by  exciting 

causes  which  are  calculated  to  increase  this  lack  of  correspondence  by  waj  of 
the  vasomotor  system.  In  my  view,  this  theory,  seductive  from  many  points 
of  contact  between  the  semiology  of  the  hemicranic  paroxysm  and  that  of  the 


460  LECTURE    XXX 

brain  pressure  syndrome,*  is  not  sufficiently  well  founded  for  my  acceptance 
of  it.  Rather  do  I  confess  myself,  with  the  great  majority  of  neurologists, 
of  the  opinion  that  the  migraine  attacks  depend  upon  vascular  spasms  in  the 
brain,  that  is,  the  hemicrania  represents  a  vaso-constrictor  neurosis;  no  other 
theory  can  explain  so  satisfactorily  the  fleeting  focal  symptoms  (henupareses, 
aphasia,  hemianopsia,  etc.),  the  accompanying  paresthesias;  the  combination 
with  vasomotor  angina  pectoris,  "dead  fingers,"  etc.  Also,  the  ophthalmo- 
scopic findings  of  Sicgrist  and  Antonelli  may  be  referred  to.  Further  there 
are  observations  in  which  thromboses  of  the  brain  vessels  and  foci  of  softening, 
occurred  after  unusually  severe  attacks  of  migraine.  The  attempt  of  Du  Bois- 
Reymond  and  Mollcndorf  to  differentiate  a  vaso-constriction  and  a  vaso-dilata- 
tion  migraine  is,  on  the  other  hand,  to  be  considered  a  failure  ;  from  the  investiga- 
tion of  vasomotor  neuroses  we  know  so  much  about  sharply  localized  vascular 
spasms,  that  it  does  not  do  to  deduce  from  the  different  condition  of  the 
arteries  of  the  integument  in  "white  migraine"  and  "red  migraine,"  that  the 
condition  of  the  brain  vessels  must  coincide  with  that  of  the  superficial  vessels. 
That  autointoxications,  hormonological  anomalies,  etc.,  are  to  be  held  respon- 
sible for  the  periodic  setting  up  of  migraine  attacks,  is  often  assumed ;  but  the 
nature  of  the  substances  coming  into  question  is  still  entirely  obscure. 

Prognosis  and  Treatment 

In  genuine  migraine,  fatal  complications,  as  we  have  indicated,  are  most 
extreme  rarities,  so  that  the  prognosis  as  to  life  is  in  general  quite  favorable. 
As  to  recovery,  a  somewhat  reserved  standpoint  is  proper,  since  not  all  casea 
recover  in  later  life,  as  is  the  rule.  Namely,  ophthalmic  and  ophthalmoplegic 
migraine  may  be  characterized  by  great  obstinacy.  Fortunately,  however, 
from  the  point  of  view  of  treatment,  we  do  not  stand  helpless  against  migraine, 
rather  in  the  great  majority  of  cases  are  we  able  to  favorably  influence  its 
course. 

As  a  general  rule  applicable  to  all  migraine  patients,  it  may  be  stated  that 
a  bland,  predominantly  lacto-vegetarian  diet,  with  frequent  taking  of  small 
portions  of  nourishment  between  the  principal  meals  (see  Lecture  XXVIII, 
page  434)  and  abstention  from  alcoholic  beverages,  exercises  a  favorable  in- 
fluence upon  the  number  and  intensity  of  the  paroxysms.  A  distribution  of 
work  and  rest  as  rational  as  possible,  avoidance  of  all  excesses,  treatment  of 
constipation,  regular  exercise  in  the  open  air,  generous  vacations  spent  when 
possible  in  the  mountains,  are  further  important  prophylactic  measures. 

Under  medicinal  treatment  are  to  be  mentioned  in  the  first  place,  occa- 
sional courses  of  bromides  recommended  by  Charcot,  Grilles  de  la  Tourette, 
Mobius,  S.  Auerbacli  and  others,  in  which  several  times  a  year  for  several 
weeks  at  a  time,  potassium  bromide  raised  to  a  dose  of  from  3.0  to  6.0  grammes 
a  day  and  then  gradually  reduced,  is  given.  More  efficient  than  the  simple 
alkaline  bromide  appears  to  me  the  following  combination  recommended  by 
Mendel:  for  20  days  at   a   time  the  patient  takes  every  morning  in   a  large 

*  Headache,  vomitinj;,  slow  pulse. 


MIGRAINE  461 

cup  of  hot  orange  flower  tea,  a  powder  of  the  following  composition:  I!  Sodii 
bromide,  2.5  (gr.  35);  sodii  salicylat,  0.25  (gr.  4);  aconitini,  0.0001  (',,, 
mg.  =  Vfiiio  grain).  For  prolonged  treatment,  the  extract  of  cannabis  indica 
is  especially  suitable;  of  this  0.015  (gr.  ]-,)  is  given,  best  every  evening  for 
several  months.  The  pil.  cannabime  comp.  which  I  have  recommended,  has 
also  been  found  useful  by  Curschmann  and  others.  When  necessary,  the  dose 
of  hashish  can  be  doubled  or  tripled  without  hesitation.  The  administration 
of  ext.  cannabis  ind.  in  a  maximum  dose  to  cut  short  the  attack  (  1J  Pastae 
guaranae,  0.25  (gr.  iv)  ;  caffein.  citrat,  0.05  to  0.1  (gr.  %  to  1%)  ;  extract 
cannabis  ind.,  0.1  (gr.  1%  in  capsule),  however,  sometimes  produces  un- 
pleasant symptoms.  Here  the  different  anti-neuralgics  (see  pages  57  and  59) 
are  to  be  preferred,  particularly  pyramidon,  migranin,  trigemin,  aspirin,  or  the 
so-called  ''mixed  powders";  among  the  last  I  would  mention  specially  the 
standard  recipe  of  Kraift-Ebing :  IJ  Past,  guaran.,  0.2  (gr.  3)  ;  caffein  citrat., 
0.06  (gr.  %o)  ;  codein  phosph.,  0.02  (gr.  %) ;  phenacetin,  0.5  (gr.  7/4); 
further,  the  equally  efficient  Edingcr's  formula:  Past,  guaran.,  0.3  (gr.  4%)  : 
antipyrin,  0.5  (gr.  7%)  ;  caffein.  citrat.  0.02  (gr.  Yz)  ;  2  powders  at  an  in- 
terval of  an  hour  as  needed.  Since  the  patients  are  only  too  much  inclined  to 
misuse  "headache  powders,"  the  taking  of  them  except  for  actual  severe  pain 
must  be  firmly  opposed.  Alternation  between  the  several  drugs,  in  order  to 
prevent  habit  formation  and  the  patient's  raising  the  dose  on  his  own  respon- 
sibility, is  greatly  to  be  recommended.  Only  in  very  rare  cases  is  it  necessary 
to  use  the  certainly  acting  morphine,  for  particularly  severe  attacks  (status 
hemicranicus).  Of  course  the  patient  should  never  be  given  the  syringe  and  the 
solution;  do  not  be  afraid,  however,  to  give  a  large  dose;  from  less  than  0.015 
(gr,  74)  a  prompt  action  is  not  to  be  expected.  We  need  not  order  the  pa- 
tient during  the  attack  to  lie  quiet  with  loosened  clothing  in  a  darkened  room 
from  which  all  noises  are  excluded  ;  he  does  this  himself  when  it  is  necessary. 
Also,  the  patients  know  better  than  the  physician  whether  cold  or  warm  com 
presses  to  the  head  suit  them  best.  As  a  prophylactic  against  the  paroxysms, 
however,  I  recommend  daily  application  of  very  hot  compresses  to  the  fore- 
head for  a  quarter  of  an  hour  after  going  to  bed.  This  is  an  adequate  sub 
stitute  for  the  hot  forehead  douches  recommended  by  Carron  dc  la  Carridre 
(which  are  in  practice  quite  complicated).  From  electrotherapy  (only  trans- 
verse galvanization  through  the  head  comes  into  question),  on  the  other  hand, 
not  much  is  to  In'  expected.  In  order  to  prevent  the  "vomiting  on  an  empty 
stomach,"  the  patient  should  be  urged  to  take  some  food  during  the  attack; 
milk,  to  which  strong  black  tea  has  been  added,  is  usually  besl    tolerated. 


THE   COPYRIGHTS    01    THIS    BOOK,    IN     Ml.    ENGLISH    SPJ    IKING    COl    NTRIES, 
\i;l     OWNED    BY    REBMAN    COMPANY,    MEW    YORK. 


Index  to   Authors 


Abadie,  177,  352 

Ac-hard.  257 

Addison,  34.5,  362 

Airy,  458 

Alzheimer,  85,  221,  231,  397 

Anton,  321 

Antonelli,  158,  460 

Apelt,   187.   337 

Aran.  107 

Aran-Duchenne,  1 I  5 

Aretaeus,  454 

Argyll-Robertson,  183,  191,  202 

Annauer,  1 17 

Aschaffenburg,  451 

Auerbach,  242,   102,   158,   159,  460 

Babinski,  6,  in.  134,  136,  147,  163,  214,  307, 

308,  140 
Ballet,  89.  352,  356 
Baranv.  36 
Bardenheuer,  1 5 
Barensprung,  317,  385 

Basedow,  315 

Bastian,  272 

Baudoin,  61 

Baumann,  353 

Bayle,  2t)<),  201 

Beard,  K)5,  H9,   120,  131,  435 

Bechterew,  52.  136,  163 

Bell,  7-' 

Benda,   366 

Benedikt,  72,  352 

Berger,  157 

Bergeron,  so.  442 

Bergman  n,  295 

Bergonie,  248 

Bernhardt,  I-'.  112 

Biedl,  363 

Bienfait,  353 

Bier,  254 

Biernacki,  177 

Biervliet,   W0 

Binswanger,  231,   232,  396 

Blrcher,   !■'...   160,  341,  381,  402 

Bircher,  H.,  341,  35? 

Blttorf,   363.  361 

Bloch,  220,  379 

Blocq,  89 

Blumenthal,  356  , 

Boas,    H9 

Bolk,  315 

Bonhoffer,  310 

Bonn,  379 

Bonnet,  52 

Borchardt,  313 

Bonrneville,  343,  393 

Bramann,  3_'i 

Bramwell,   125 

Braun,  198 

Brissaud,  52,  6T,  178,    '  89,  S67,  I  '■* 


Broadhurst,   B2 

Broca,  272 

Broedel,  225 

Brosius,  165 

Brosse,  405 

Bronardel,  13 

Brown-Sequard,  65,  139,  156,  161,  362 

Bouchard,  264 

Brims.  138,  111    310,  379 

Bryson,  349 

Bumke,  202 

Burghardt,  356 

Buschan,  340 

Calmeil,  457 

Campbell,  385,  386 

Carron  de  la  Carriere,  461 

Cassirer,  185,  303.  376,  377 

Charcot,  2.3.  88.  105,  111,  117.  132,  135,  138,  139, 

148,   178.    194,  226,  256,  264,  358,  399,    ins. 

410,  422.  436,  443.   414,    157,    160 
Cheyne-Stokes,  180,  267 
Chvostek.  76 
C'lnnct,  89,  90 
Coester,  3 
Cooper,  Astley,  501 
Corning,  253  " 
Corti,  33 
Corvisart,  71 
Cramer.    106.    118 
Cullen,  104 
Curschmann,  95,  :^>K  373.  371.  376.  379.  380,  156, 

16 1 
Cushing,   334,  367 
Cuvier,  318 

Damsch,  325 
Dana.  309 
Dax,  273 

Dean,  317 

Dejerine,  3,  113,  115,  124,  131,  136,  137.  Is;,,  210, 

226,  878,  279,  328,  373,  124 
Demange,  .'.'7 
Desvault,   L84 
Dietschy,  379 
Dieulafoy,  .ir.ii,  i.-,i 
Dinkier,  193 
Dobrschansky,  208,  211 
Don.illi.   .'I  I,  397 
Down,  339 
Dreschfeld,  310 
Dubief,  89 
Dubini,  so 

Dubois,  67.  105,   130,  131,   1 1» 
I)n  Bois-  Fteymond,   166,   160 
Duchenne,  73,  107,  lis.  [64,  17.'.  .'37 
Durdufl,  353 
DUring,  378 
Durkheimer,  89 
Durand-Fardel,  .'.'7 


468 


404 


IXDEX    TO    AUTHORS 


Eckert,   -'.".1 

Edinger,  4,  44,  128,  160,  431,  458,  461 

Ehrlich,  44 

Eiehhorst,  12,  134 

Eiselberg,  367 

Elischer,  82 

Ellis,  211 

Eisner,  317 

Enada,  193 

Enriquez,  352,  356 

Eppinger,  371 

Erb,  9,  50,  76,  94,  97,  98,  104,  111,  164,  165, 

214,  226,  325,  375 
Erdheim,  75 
Erlenmayer,  222 
Esquirol,  202 
Eulenburg,  91,  193,  352 
Ewald,  358 

Falret,  200,  427 

Faure,  184 

Feindel,  67 

Fere,  457 

Filelme,  353 

Fischer,  211 

Flajani,  345 

Fla'tau,  456,  457,  458,  459 

Flexner,  242,  243,   252 

Forster,  116,  198,  335,  441 

Forli,  316 

Fothergill,  17 

Fournier,  164,  184,  186,  320 

Frank,  318 

Franke,  198 

Frankl-Hochwart,  77 

Frenkel,  128,  197 

Freund,  137,  330,  437 

Freud,  279,  331,  332,  428,  419,  450 

Frey,  127,  340 

Friedlander,  211,  264 

Friedreich,  73,  123,  213,  352,  384 

Frund,  213 

Fuchs,  187 

FUrstner,  202 

Gartner,  348 

Galen,  38!),  154 

Galton,  35 

Ganghofner,  330 

Ganser,  445 

Garre,  352 

Gaupp,  202 

Gauthier,  89 

Gendrin,  447 

Generali,  75 

Gerhardt,  149,  414 

Gerlier,  93 

Gersuny,  383 

Gilles  de  la  Tourette,  400,  460 

Goppert,  250 

Goldflam,  94 

Goldscheider,  197 

Gowers,  126,  128,  356 

Graef'e,  317 

Grasset,  226,  356,  455 

Gratiolet,  295 

Graves,  315 

Griesinger,  427 

Gull,  358 

Gutzmann,  283 


Haegler,  382 

Hagenbach,  75,  92 

Hanau,  366 

Hansen,  147 

Harbitz,  245 

Hartenberg,  458 

Head,  7,  385,  386 

Hebra,  380 

Heilbronner,  284,  285,  401 

Heine,   159,  235 

Heller,  37S 

Helmholtz,  318 

Henoch,  80 

Hess,   371 

Hessing,  248 

Heubner,  251,  255,  264,  335 

Heuschen,  458 

Hippocrates,  397,  405,  427,  436 

His,  459 

Hnche,  308 

Hndskins,    401 

Hoffa,  248,  335 

Hoffman,  77,  105,  107 

Holmes,  308 

Homburger,  223,  230,  233,  349 

Horner,  146,  149,  371 

Huchard,  225,  350 

Hiibscher,  248 

Hughes,  73 

Hunt,  37,  309 

Huntington,  80,  84,  128 

Hunziker,  381 

Ibrahim,  332 
Inada,  232 
Iselin,  75,  95,  288 

Jaboulay,  198,  357 
Jaccoud,  417 
Jacquet,  91,  420 
Jakowenko,  82 

.la net,   428 
Jauregg,  211,  343 
Jendrassik,  174 
Jochmann,  252 
Joffroy,  148,  205 
Jolly,  91,  458 
Joseph,  381 
Josue,  222 
Jusgen,  209 

Kassowitz,  69,  79 

Katzenstein,  310 

Kleist,  285 

Klieneberger,  303 

Klipell,  185 

Klippel,  231,  364 

Klumpke,  17,  147 

Knapp.  193 

Knoblauch,  91,  207,  208 

Koch,  300,  365 

Kocher-de-Quervain,  70 

Kocher,  321,  350,  353,  357,  358,  361,  402 

K.'inig,  198 

Kohler,  145 

Kolle,  252 

Roller,  407 

Kolliker,  369 

Kollovits,  128,  412 

Kownlewsky,  340 


INDEX    TO    AUTHORS 


465 


Kraepelin,  201,  205,  207,  210,  218 
Krafft-Ebing,  200,  204,  210,  461 
Krause    (Fedor),   61,   288,   312,   313 
Kron,   193 
Kunn,  13ii 
Kussmaul,  202,  419 
Kutscher,  252 

Laliarraque,   156 

Laborde,  71 

Ladame,  231,  393 

Laniy,  243 

Landry,  42,  159 

Landsteiner,  243 

Lane,  339 

Lange,  60,  61 

Lannelong,  339 

Lanz,  352,  3S6 

Lasegue,  51,  53,  60,  448 

Launois,  393 

Lechner,  410 

Legrand  du  Saulle,  394,  399,  427 

Lupine,  356 

Leredde,  210 

L6ri,  186 

Lctulle,  179 

Leube,  350,  379 

Levaditi,  241,   2i2,  243 

Levico,  89 

Levy,  61,  252,  343,  350,  352,  360 

I.ewin,  37!) 

i  .ru  is,     .'  I  .' 

Leyden,  128,  197 
Lichtheim,  279 

Liepman,  27K,  279,  283,  281,  285 
Little,   116,  327 
I.ivcing,  457 

In,- 1,.    7S 

Long,   133 
Louril,  315 
Luciani,  315 
Lundborg,  95 
Lussona,  :il  1 

\l.i  allnm,  78,  356 
Macewen,  313 
Mackenzie,  351 
Madelung,  368 
Magendic,  310 
Mankowsky,  356 
Mann,  77 
Mannheim,  350 

Man/,    ."III 

Marburg,  368 

Marie,  105,  128,  133,  327,  231,  342,  .'73.  320, 

366,  367 
Marina,  104 

Marinesco,  95,  98,  I  16,  27:! 
Mil.  ma,  3ii  I 
Maxquelle,  89  ' 

Med  in.    159,    -'35 
Meige,  67.  381,    159 
Meltzer,  320 

Mendel,  136.  163,   161,  193,  225.  256,    WO 

\l rv.   138,  217 

Mignot,  202 

Mikulicz.  321 

Mingazz.ini.    198,    -'.'II.    311 

Minkowski,  :t 
Minkowsky,  1  I  -' 


Miura,  93 

Mobius,   165,  317,  439,  353,  356.  457,  458,  469 

Mollendorf,  460 

Moliere,  405 

Monakow,  263,  273,  279 

Moore,  211.  382 

Morgan,  401 

Morris,  382 

Morse,  73 

Morton,  53,  459 

Morvan,  146 

Moussu,  75 

Miiller,  126,  136,  193.  232,  236,  391 

Miiller,  A.,  459 

Miiller,   Fr.,  350,  351 

Munk,  316 

Muskens,  391 

Kageli,  425 

Nagelschmidt,  248 

Kagy,  (i  I 

Naka,  202 

Neisser,  293 

Xe   Kam,  376 

Netter,  241 

Nensser,  363 

Nicoladoni,  248 

Nissl,  1S7,  244.  338 

N'ognehi,  187,  211.  243 

Nonne,  1  11,  186,  187,  202,  209,  213,  220 

NorstrSm,  458 

Nothnagel,  225,  309,  373 

Nns.sliauin,  73 

Obersteiner,  171,  203,  205,  207,  218,  301 

Ollivier  d' Angers,  143 

Oppenheim,  4,  43,  45,  47,  52,  66,  72,  86,  89,  96, 

121.    126,    133.    136,    139,    112.    163,    179.    IS5. 

191,    2(19.    2211,    227,    212,   247.   303.   3111.   313. 

317,   351,   374,  396,  401,   410,    411,    115.    127, 

417,   156,   157 
Ord,  358 
Ostwaldt,  61 

Pal,  126 
Pansini,  316 
Parchappe,  200 
Parinaud,  136.  138 
Parisot,  202 

Parkinson,  86,   116 

Paxal,   IBS 

I'avr.   321,  313 

I'critz,    .'.-..'.    158 

I'ilcz.   211 

Pineles,  75.  76,  77,  78,  310 

Piorry,    158 

Pitres,   i!'1 

Placzek,  31:'. 

Pollack,  .'93 

Pool-Schlesinger,   76 

Pouchet,  -':'•-' 

Qudnu,  51 

Quervain,  75,  293 

(Quincke,  253,  321.  381,  382,   159 

Qulnquaud,  63 

lianzier.    I'.'. 

Ravaut,  181 

Ravmond,   198,  l-'..   I"'..  350,   I  >.   160 


466 


INDEX    TO    AUTHORS 


Raynaud,  373 

Recklinghausen,  5 

Redlich,  89,  171 

Regnaiilt,  64 

Reiehardt,  202,  288,  459 

Reisinger,  383 

Reitmann,  379 

Remak,  3,  40,  314 

Rethi,  136 

Reumdht,  165,  193 

Reverdin,  358 

Richet,  399,  444 

Rinne,  34,  185,  441 

Robertson,  183 

Rodari,  196 

Roemer,  241,  243 

Romberg,  164,  173,  191,  235,  383 

Rosegger,  343 

Rosenbach,  430 

Rosenthal,   187 

Rossolymo,    134 

Roth,  42 

Rothmann,  243,  310,  315 

Roussy,  89,  90 

Roux,"  129 

Runge,  319 

Russell,  310 

Rust,   163 

Rynberk,  315 

Sachs,  278,  339,  337 

Sanger,  220 

Sainton,  320 

Salle,  159 

Sander,  89 

Scarpa,  198 

Sehachnowiez,  93 

Scheel,  245 

Schiff,  185 

Schleich,  60 

Schlesinger,  146,  325,  373 

Sehloesser,  61,  67 

Schmidt,  290 

Scholz,  350 

Schrameck,  202 

Schuller,  290 

Schulthess,  248 

Schultze,   112,   141,   145,   149,   246,   373 

Schuster,   193 

Schwabach,   185 

Schwann,  6 

Secretan,  382 

Seeligmueller,  47,  217 

Senator,  128 

Srvigne,  405 

Siegrist,  458,  460 

Siemerling,   185 

Smith,   Frank,  3 

Soea,  124 

Somma,  320 

Sonden,  420 

Splelmeyer,  209,   370.  338 

Spillmann,  192 

Stahelin,  350,  379,  381 

Stellwag,  347,  348 

Sternberg,    367 

Stewart,  281 

Stierlin,  419,  440 

Stintzing,  10 

Stolting,  138 


Strumpell,  78,  112,  113,  115,  133,  242,  246, 

303,  329,  365 
Stuart,  308 
Swift.   211 
Sydenham,  82 

Talma,   91 

Tay,  337 

Tedeschi,  353 

Theohari,  3 

Thiemich,  77 

Thiersch-Witzell,   61 

Thirial,  377 

Thomas,  124,  129,  133,  190,  315,  316 

Thomsen,  90 

Tigerstedt,  420 

Timaeus,  436 

Tissot,  403,  456 

Tobias,  164,  193 

Tooth,  105 

Toulouse,  399 

Trombert,  373 

Trommer,  415 

Trousseau,  48,  75,  76,  349,  350,  399,  331,  351,  393 

Tuczek,  307,  208 

Uhthoff,  138,  184 
Ulrich,  400 

Valleix,  46,  47,  49,  59,  60,  67 

Yal  Sinestra,  89 

Vassale,  75 

Vauban,  457 

Yigouroux,   349 

Villiger,  151 

Virchow,  320,  339 

Vogt,  329,  338,  340 

Volt,  420 

Vorschiitz,  254 

Yulpian,  132,  262 

Waller,  5,  157 
Wanner,  384 

Wassermann.  140,  164,  182,  187,   209,  252 

Weber,  35,  185 

Weigert,  95,  126 

Weir-Mitchell,  40,  382,  435.   1 53 

Werding,  107 

Wernicke,  273,  378,  379,  280,  303 

Wernitz,  324 

AVertheim-Salomonson,  16 

Westenhoffer,  248 

Westphal,  141,  174,  191,  200,  207 

Weygandt,  340 

Wickman,  242,  245 

Widal,  187 

Wiesel,   363 

Wille,  207 

Williamson,  246 

Willis,  36 

•Windscheid,  222 

Wolff,  201,  280 

Wollenberg,  308 

Wunderlich,  194 

Wyss,  318 

Zabludowski,  73 
Ziehen,  86,  210,  309 
Zupnik,  252 


General  Index 


A  bad  ins  tabes,   177 
Abdominal  reflex,  8 
Abducens,  31,  39 
paralysis,  324 
Abiotrophy,  1 28 
Abiinisin,  3s7 

Abscess  of  the  cerebellum,  31 2 
Abscess   of  the   spinal   cord,   158 
Accessory  nerve,  37 
Accident'  hysteria,  424,  446,  450 
Accident  neurasthenia,  424 
Accidental  autopsy  findings,  301 
Accoucheur's  hand,  75 
Achilles    reflex,   S 
Achillodynia,  S3 
Achondroplasia.  341 

Acrocyanosis  chronica  anoesthetica,  377 
Acromegaly,  3<i."),  3<;7 
Acroparesthesia,  373 
Actinomycosis,  289 

Acute  apoplectic  bulbar  paralysis,  2(15 
Acute  ataxia,  192 

Acute  atrophic  spinal  paralysis,  -+<> 
Acute  bulbar  paralysis,  303 
Acute  decubitus,  262 

Acute  dilatation  of  the  heart,  115 
Acute   hemorrhagic   encephalitis,   303 
Acute   infectious  disease  of   the  central   ner- 
vous system,  235 
Acute  poliomyelitis  of  adults,  _> Hi 
Acute   post-operative   thyroidism,   352 
Adenoma,  289 
Addison's  disease,  362 
Adiadochokinesis,  308 
Adipositas  dolorosa,  387 
Adipo.so  genital  degeneration,  3fi8 
Adrenal  insufficiency,  262 
Adrenalin  glj  cosuria,  371 
Aerophagia,   143 

Affect    spasms.    I  15 

Agenesis  eorticalis,  337 
Agenitalism,  368 
Ageusia,  30,  37 
Agnosia,   272,   286,  321 
Agoraphobia,   127 
Agraphia,  280,  285 
Akinesia   algera,   139 
Ucoholic  polyneuritis,  12 
Alcoholism,  :'.'.  39,  13,  IS,  63,  7.'.  75,  128,  158, 
■I  :.  :io::.  31  I.  317.  :;."i.  :iss.    I'm,,   i.'i;,   |  »i 
Alexia.   275,    -"-'I 

Allocheiria,  170 
Allorhythmia,  115 
Alterations  of  handwriting, 

Alternating    facial    hemiplegia.    .'70 

Alternating  oculomotor  hemiplegia,  270 

Amaurosis.   26,    158 

Amaurotic  idiocy,  3.37 


Ambidextrism,  336 
Amblj  opia,  26 

Ambulatory   automatism,  394 

American  nervousness,   l_'| 

Americanization.   421 

Amimi  i.   280 

Amnesic  aphasia,  206,  279 

Amputation   lines,    I  lo 

Amusia,  280 

Amyotrophic  lateral   sclerosis,  116,  147 

Anacusis,  33 

Anadrosis,  9 

Analgesia,    li 
Anal  reflex,  S 
Anaphrodisia,    1J| 
Ancle  clonus.  1 1  I.  1  17,  163 
Anemia  of  the  brain,  304 
Anesthesia,  (i,  28 

circular,  140 

disseminated.   1 10 

geometrical,   110 

insular.    11(1 

universal,  410 
Angina  pectoris,  373,  415 
Angio  neuroses,  369 
Angio-paralvtic   migraine,   456 
Angiospastic  dysbasia,  37 1 
Angio-spastic  migraine.  156 
Angio-spastic  symmetrical  gangrene.  37. 
Anidrosis,  372 
Anisocoria,  183,  202 
Anomalies,  of  metabolism,  350 

of  the  external  ear,  394,    107 
Anorexia,    I  13 
Anosmia,  26 
Anospinal  center.  155 
Ansa   hj  poglossi,  3s 
Anterior  polj  neuritis.   235 

Anticipatory   heredity,  si 
Antineuralgics,  57,  59,   Iff", 

Antispasi lica,  <>T 

Anxietas  tibiarum,   1 1 J 
Anxiety  neurosis,  t.s 
Ape  hand,  108,  1 18,  l  ID 
Aphasia.  205,  -'7.'.  279,  158 
i  oi  Heal  motor.  276 
Cortical   sensory.   .'77 

of  single  senses.  27i» 

subcortical   motor,  277 
Wernicke's,  .'77 
Apoplectic  attack, 
\  [loplectic  ci  st,    16  I 
Apoplectic  scar,  264 
Apoplectiform  alcoholic  paralysis,   \2 

Apoplexie   f Iroj  anti 

Apoplexy,    162 
Ingravescent,  -'<i7 

I  r.  ,ii,,   271 


KIT 


468 


GENERAL    INDEX 


Apraxia,  205,  272,  283 

ideo-kinetic,  284 

transcortical,  284 
Arachnitis  chronica,  201 
Aran-Duchenne  type  of  progressive  muscular 

atrophy,  107 
Arc  de  cercle,  -145 
Areflexia,  8,   173 

Argyll-Robertson  symptom,  183,  202,  217 
Argyria,  194 
Arhythmia,  415 
Arithmomania,  428 
Armauer-H arisen  bacillus,  147 
Arsenic,  4,  194,  356 
Arsenical   polyneuritis.    1:> 
Arteria  sulco  commissuralis,  213 
Arteriosclerosis  of  the  nerve  centers,   222 
Arteriosclerosis  of  the  spinal  cord,   286 
Arteriosclerotic  age.  22S 
Arteriosclerotic  changes  in  the  brain  vessels, 

263 
Arteriosclerotic  neurasthenia,  222 
Arteriosclerotic  neuritides,  42 
Arteriosclerotic  paresis,  232 
Arteriosclerotic  pseudo-paralysis,  232 
Artery  of  cerebral  hemorrhage,  25(i 
Arthogryposis,  78 
Arthritic  diathesis,  455 
Arthrodosis,  107,  .Ms 
Arthropathies,  178 
Ascending   degeneration.   213 
Asphyxie  locale  symmetrique,  375 
Associated  movements,   111,  261,  330 
Astasia-abasia,  447 
Astereognosis,  295 
Asthenopic  troubles,  411 
Asthma,  416 
Vstigmatism,  395 
Asymbolia,  280,  283 
Asynergy,  7 

Asystematic  affections,  166 
Ataxia,  7,  124,  172,  307 

cerebellar,  242 
Athetoses,  85,  331 

double,  85,  332 
Athvreosis,  358 
Aloiiia,  8 

Atrophic  excavation,  27 
Atrophies  from   overuse,  3 
Atrophy,  individual,  108 

from  disuse,  1 13 

of  nurslings,  302 

of  the  papilla,  205 
Atypical  forms  of  multiple  sclerosis,  139 
Atypical  hemiplegias,   270 
Auditory,  33 

agnosia,  286 

aphasia,  280 
Aura  cursatoria,  389 

sensory,  75 
Auriculo  temporal  point.  48 
Automobilist  cramps,  72 
Autonomic  nervous  system,  369 
Autosuggestion,  431 
Avaricious  concepts,  425 
Avertissements  sans  frais,  226 
Aztec  type,  339 

Babinski  phenomenon.  111.  124,  163,  226 
Babinski  reflex,  212,  261.  300,  330 


Babinski  toe  phenomenon,  390 

Balkenstich,  321 

Ballet-dancer's  cramps,  72 

Balneotherapy,  136,  147,  196 

Barany's  test",  36,  309 

Basedow's  disease,  345,  364 

Basedow's  goiter,  346 

Basedoi'jified  goiter,  352 

Bathyanesthesia,  10 

Bathyesthesia,  7,  156 

Bathyhypoesthesia,  10 

Bechterew's  sciatic  phenomenon,  52 

Bed-sore,  321 

Bell's  paralysis,  31 

Being  possessed,  388 

Being  seized,  388 

Benign  hypo-thyroidism,  360 

Beri-beri,'  5,  13' 

Biceps  reflex,  8 

Bier's  boxes,  57,  380 

liii  nmrk's  symptom,  177 

Bird  head,  339 

Blahals,  445 

Blastophthoria,  128,  396,  407 

Blepharoclonics,  (Hi 

Blepharospasms,  66 

Blindness   for  letters,  280 

Blood  changes,  350 

Blood  pressure,  276 

Bloodletting,  267 

Bloody  nerve  stretching,  60 

Blowing  smoke,  262 

Blue  oedema,  443 

Bone  reflex,  8 

Bonnet's  phenomenon,  52 

Bounding  mydriasis,  202 

Bounding  pupils.  183 

Bouton  diaphragmatique,  49 

Brachialgias,  50 

Brachial  neuralgias,  50 

Brachial  plexus  paralysis,  16 

Braehioradialis,  14 

Brachium  conjunctivum,  destruction  of,  310 

Bradycardia,  415 

Brain,  abscess,  297 

disturbances  of  circulation  in,  288 

inflammations,  288 

massage,  402 

pressure,  289,  321 

swelling,  288 

tumor,  288 
Broca's  aphasia,  273,  276 
Broca's  center,  273 

Broca-W ernicke  localization  theory,  273 
Bromide  therapy,  399,  460 
Bronze  diabetes,  364 
Bronzed  skin,  363,  364 
Brown-Siquard  paralysis,  156 
Brown-Sequard    symptom-complex,     139,     155, 

161,  219 
Bulbar  paralysis,  122 
Burdach's  column,  168 

Cachexie  pachydermique,  358 
Cachexia  strumi-priva,  358,  361 
Caisson  paralysis,  159 
Caloric  nystagmus,  309 
Capsular  hemiplegia,  256 
Caput  obstipum  spasticum,  68 
Caput  progeneum,  407 


GENERAL    INDEX 


469 


Carcinoma,  289 

Carioptosis,  415 

Cardio-vaseular  disturbances,   111 

Cardio-vasomotor  neurasthenia,  418 

Carrefour  sensitif,  257 

Catalepsy,  1 15 

Cellist's  cramps,  72 

Center  for  the  memory  of  sounds,  272 

Central  gliosis  of  the  spinal  cord,  143 

Central   pain,  295 

Cephalaea,  454 

nodular,  459 

rheumatie,  459 
Cephalagia,  454 
Cephalones,  320 
Cerehellar  affections,  306 
Cerebellar  asynergy,  307 
Cerebellar  attacks,' 309 
Cerebellar  ataxia,  307 
Cerebellar  fits.  309 
Cerebellar  heretio  ataxia,  128 
Cerebellar  hypotonia,  308 
Cerebello-pontine  seizure,  309 
Cerebellum,  agenesis  of,  315 

atrophies  of,  315 

diseases  of,  306 

hemorrhages  of,  314 

infectious   diseases   of,   316 

softenings  of,  314 

tumors.  506 
Cerebral  atrophy,  261 
Cerebral  embolism,  265 

Cerebral  glosso-pharyngo-labial  paralysis,  229 
CVn-bral  hemiplegia,  256 
Cerebral  hemorrhage,  255 
Cerebral  meningitis,  328 
Cerebral  monospasm,  293 

Cerebral   muscular  atrophy,  33:) 
( lerebral    neurasthenia,  1 1^ 
Cerebral  porosis,  227 

Cerebral  softening,  156 
Cerebral  vomiting,  290 
Cerebro-cardial  neuropathy,   to.'. 
Cerebro-cerebellar  atrophy,  315 
Cerebrospinal  atrophy,  315 

fluid,  187 

syphilis,  .'15 
Charcot's  disease,  117 

extension  type,  88 
Charcot-Marie  type  of  progressive  muscular 

atrophy,    105 

Chemical  chamisole,  -'I  I 
Cheyne-Stokes'  respiration,  267,    !92 
Chloride  of  gold,  [94 

Cholesteal a.  289 

Chondrodystrophy,  foetal,  341 
Chondroma,  289 
Chordoma,  289 
Chorea,  310.  XU 

bodies,  82 

electric,    I  I  .' 

gravidarum,  s-'.  85 
hereditary .  s' 
Huntington' i,  84 
hysterical,  s" 
major,  80,  L4S 

minor.  80 

Mnllr's.   83 

paral]  tica,  82 

sancti  viti.  80 


Chorea.  Sydenham's,  80 
Choreiform  diseases,  80 

movements,  350 
Chromaffin  system,  M2 
Chronic  anterior  poliomyelitis,  2\:t 

form  of  neurotic  oedema,  381 
( 'hvostt  k'.t  sign,  77 
Cigarmaker's  cramps,  72 
Ciliary  neuralgia,  17 
Cilio-spinal  center,  146 

Circulatory  disturbances  of  the  brain.  304 
Circumduction,  113,  260 
Circumflex   nerve  paralysis,   19 
Cirrhosis  of  the  chorium.  379 
Clarinetist's  cramps,  72 
Claudicatio  intermittens,  125 
Claustrophobia,  427 
Clavus  hystericus,  138 
Clawfoot.  23,  116 

Claw  hand,  20,  108,  118,  145,  149,  239 
Cleft  palate,  407 
Cleido-cranial  dysostosis,  320 
Clitoris  crises,   1st 
Clonograph,  413 
Clonus,    111 
Clouding  of  consciousness,  394 

postconvulsive,  394 

postepileptic,  394 

postparoxysmal,  394 

preconvulsive,  394 

preepileptic,  391 

preparoxysuial,  394 
Clownism,  1 1 1 
C'ocainism,  396 
Coccygodynia,  53 
Cochlear  nerve,  33 
Coin  counting,  81 
Cold   water  cures,   355 
Collateral  ganglion  system,  370 
Color  scotoma,   26 
Colored  vision.  389 
Combined  tabes,  215 
( Composite  movements,  316 
Compression  paralysis,  -' 
Compulsion  neurosis.  138 
Compulsory  crying,  13S 

laughing,    13S 

movements,  310 
Concentric  limitation   of  the  visual   Held,   WO 
Concentric  narrowing  of  the  visual  field,  -'i> 
Conci  pi    center.    J86 
Conceptual  center,  273.  .'71 
Conception  of  movement,  J06 
Concussion  of  the  brain,  263,  304 

of  the  spinal  curd,   150 

Conduction  aphasia,  279 
Conduction,  disturbances  of,  .' 

symptomatology .  >' 
Congenital,  acquired  defective  conditions,  317 

bulbar   paralysis,   ::.'i 
criminal.    In; 
muscle  defects,  324 

muscular  atony,  92 

nucle  tr  defects,  334 

ptosis.  39  i 

syphilis.  339 
Congestion  of  the  brain.  304 
Conjugate  Ae\  iation,  310 
Conjunctival  reflex,  29 
Consensual  light  reaction,  183 


470 


GENERAL    INDEX 


Constitutional  neurasthenia,  -125,  4J(i 

Contracture  diathesis,  443 

Contracture  rheumatismale  des  nourrices,  75 

Conversion,  4+9 

Cooperation  movements,  307,  310 

Coordinatory  occupation  neuroses,  72 

Corneal  reflex,  29 

Corpus  callosum,  defect  of,  337 

Corpus  callosum,  puncture  through  the,  321 

tumors  of,  295 
Cortical  alexia,  280 
Cortical  apraxia,   285 
Cortical  attacks,  218 
Cortical  epilepsy,  205,  218,  293 
Cortical  hemiplegia.  270 
Cortical  paralysis,  293 
Cotugno's  disease,  51 
Coups  de  hache,  102 
Cracked  pot  sound,  290 
Craemer  four  groups  of  phenomena,  448 
Cranial  ectopies,  321 
Cranial  nerves,  26 
Cranial  paralysis,  38 
Cranianiphitomie,  339 
Cremasteric  reflex,  8 
Cretinism,  341 

Cretinistic  degeneration,  341 
Cretinistic  idiocy,  341 
Cretinoids,  343  ' 
Crises  comitiales,  388 
Crises  noires,  180 
Crises  of  emaciation,  350 
Critical   situations,  431 
Cyclothymia,  429 
Cystic  degeneration,  227 
Cysticercus,  289 
Cysts  of  softening  of  the  brain,  328 

Dammerzustand,  394 

Daktvlographer's  cramps,  72 

Danse  de  Saint  Guv,  80 

Dead  finger,  376,  377,  441 

Deafness,  33 

Deceptive   front,  231 

Decompressive  craniectomy,    296,   312 

Defatigatio,  423 

Defective  conditions,  317 

Degenerative  neurasthenia,  426 

Degenerative  processes,  9 

Dejjerine-Lichiheim  phenomenon,  277 

Delire  du  toucher,  428 

Demarche  a  petit  pas,  229 

Dementia  myoclonica,  74 

Dementia  paralytica,  121,  205 

Dementia  praecox,  429 

Demoniacal  attacks,  445 

Dental    points,  48 

lii  ream's  disease,  387 

Derivatives,  70 

Dermatotomes,  175 

Dermographia,  250 

Dermographism,  347,  416,  441 

Dermoid  cysts,  289 

Descending  degeneration,  213 

Desire  for  excitement,    1 13 

Development  of  the  eortico-spinal  tracts,  328 

Deviation  conjuquee.  262 

Diaphragm  crises,  180 

Diaphragm  spasms,  71 

Diarrhoeas,  349 

Diaschisis,  149,  263,  273 


Diaschisis,  theory,  273 
Diathermy,  248 
Diffuse  diseases,  111 
Diplegia,  7 

facialis,  31,  94,  217 

masticatoria,  30 

spastica  infantilis,  330 

superior,  236 
Dij)  on  the  parallel  bars,  326 
Dipsomania,  394 
Direct  light  reaction,  182 
Disseminated  neuritides,  41 
Disseminated  sclerosis,  132 
Dissociated  anasthesia,  145 
Dissociated  disturbances  of  potence,  137 
Disturbances  of  menstruation,  349 
Doubting  mania,  427 

Divergence  of  the  eyeball  of  Magendie,  310 
Diver's  paralysis,  159 
Dorsal  foot  reflex,  114 
Douleurs  en  brodequin,  180 
Douleurs  fulgurantes,  180 
Douleurs  lancinantes,  180 
Drawling  speech,  203 
Drop  foot,  23 
Drummer's  cramps,  72 
Duchenne-Erb  type  of  paralysis,  17 
Duchenne-Griesinger  type  of   dystrophy,   102 
Dwarfism,  341 

Dysbasia  angiosclerotica  intermittens,  225,  373 
Dysbasia  lordotica  progressiva,  so 
Dysbasia  neurasthenica  intermittens,  412 
Dyschromatopsia,  441 
Dysglandular  symptom-complex,  345 
Dysglandular  syndromes,  367 
Dyskinesias,  63,  80 
Dyskoimesis,  410 
Dysmetria,  172 
Dysnystaxis,  410 
Dysphylaxia,  410 
Dyspinealism,  295,  368 

Dyspraxia  angiosclerotica  intermittens,  373 
Dystrophia  musculorum   progressiva,  97 
Dystrophy,  progressive  muscular,  325 
Dysthyroidism,  353 

Early  acquired  defective  conditions,  317 

Echinococcus,  289 

Echolalia,  71 

Echopraxia,  71 

Eclampsia  in  infants,  77 

Eclampsia  infantium,  388 

Ecmnesia,  445 

Elbow  clonus,  114 

Electivity,  42 

Electric  chorea,  80 

Electric  hand,  105 

Electrodiagnosis,  10,  77,  91,  95 

Electro  physiology,  10 

Electrotherapy,   55,   60,   67,   196.   217,    269,  335, 

355,  375 
Embolism  of  basilar  artery,  121,  147 
Encephalasthenia,  418 
Encephalitis,  congenital,  327 

non-suppurative,  302 

post  partum,  328 

subcortical  chronic  progressive,  232 
Encephalocele,  321 
Encephalomalacia,  256 
Encephalopathia  addisonia,  364 
Encephalorrhagia,  256 


GENERAL    INDEX 


471 


End  arteries,  256 

Endarteritis,   syphilitic,  217,  329 

Endarteritis  syphilitica  obliterans,  364 

Endocrine  glands,  315 

Endogenic  fibers,   170 

Endogenic  fields  of  the  posterior  columns,  170 

Endothelioma,  289 

Endotbeliome  en  nappe,  289 

Enophthalmus,  30,  :S71 

Enuresis,  324,  426 

Ependymitis,  317 

Epidemic  cerebro-spinal  meningitis,  218 

Epigastric  reflex,  8 

Epilepsia  convulsiva,  389 

major,  389 

non-convulsiva,  392 
Epilepsie  marmottante,  392 
Epilepsy,  242,  388 
Epileptic,  aura,  3S9 

equivalents,  393,  454 

paranoia,  395 

syncope,  393 

vertigo,  392,  393 
Epileptogenic  zone,  396 
Epithelioma,  289 
Erl>  type  of  dystrophy,  102 
Erb-Goldflam  disease,  94 
Ergotism,  373 
Erlenmeyer's  mixture,  398 
"Ersatz"  theory,  44 
Erythromelalgia,  382 
Erythromelalgic  attacks,  382 
Erythromelia,  383 
Ereuthophobia,  416 
Erythroprosopalgia,  382 
Ettnarch  bandage,  2 
Essentia!  infantile  paralysis,  235 
Etat  de  fromage  de  Gruyfere,  227 

lacunaire  du  cerveau,  227 

vermoulu,  22'i 
tftat  second,  391 
Exaggerated  perception,  H5 
Examination  neurasthenia,  123 
Exhibitionism.  394 
Exophthalmus,  346 
Exploratory   laminectomy,  162 
Extracapsular  hemiplegias,  270 
Extramedullary  tumors,  161 
Kxtra    systoles.'  415 
Extremities,  spasms  of,  71 
I  ye  muscle,  paralysis  in  Basedow's  disease, 
348 


Facial  cramps,  66 
Facial  hemiatrophy,  38  I 
Facial  hemihypertrophy,  383 
facial  hemispasm,   tS8 
Facial    nerve,  31 

Facial  paralysis,  3.  3t,  324 
Facies  myopathica,  10] 
Factitious  urticaria,   H6,   in 
Falling  sickness,  388 
Fatigue  pain,   1 1  - 
Fetichism,  ill 
Fibers  in  the  posterior  r 
Fibrillary  contractions, 
I  Hi.  ill 

tremor,    106 
twitchings,  203 
Fibroma,  289 

Finger  clonus,   11 1- 


167 
His,    I1H.    119, 


Finger-finger  test,  173 

Finger-nose  test,  173 

Flail  joint,  2:19 

Flea-bite  encephalitis,  303 

Flechsig's  bromide  opium  cure,  400 

Fliegende  Roeten.   116 

Flutist's  cramps,  72 

Foci  of  softening  of  the  brain,  328 

Forster's  operation,  335 

Forster's  type  of  alteration,  441 

Fcetal  chondrodystrophy,  341 

F'olie  du  doute,  427 

Folie  musculaire,  81 

Forced  attitudes,  310 

Formes  frustes,  346,  360 

Fothergill's   face  pain,  47 

Four  cell  baths,  374 

Four  reactions,  329 

Four  reactions  of  Nonne,  209 

F'ree  warnings,  226 

Friedri  ich's  disease,  123 

Friedreich's  foot.  125 

Frohlich's  disease,  368 

Front  olympien,  320 

Frontal  headache,  290 

Full  feeding  cure,  199 

Fully  developed  psychoses,  205 

Funiculus  cuneatus,   212 

Funiculus  gracilis,  212 

Functional  ankle  clonus,  ll:( 

Funnel  chest,  395 

Qalton's  whistle,  35 
Galvanic  irritability,   13 
Galvanic  vertigo.  36 
Gampsodactvlism,    107 

i  langliated  cord,  369 

Oanser's  symptom,   115 

Gasserian  ganglion,  extirpation  of,  61 

Gastralgokenosis,  1 19 

Gastric  crises,   iso,  196 

Gastrorrhea,  acid.  1 16 

General  hyperesthesia,  405 

General  rigidity,  :i'M 

Genickstarre,  249 

Genital  disturbances,  186 

Genu  recurvatum,  173 

Gephyrophobia,  106,  127 

t  ;>  rating's  method,  \\*:\ 

Giantism,  367 

Girdle  pain,  180 

Glandula  pituitaria,  366 

Glanzauge,  346 

Glassworkers,  2 

Glioma,  J88 

Gliosarcoma,  288 

Gliosis,  spinal.    ISO 

Glistening  eye.  316 

Globulin    reaction.   1S7 

( llobus  hj  stericus,   1 13,   1 1 1 

Glossolabial  hemispasm,   lis 

Glossoplegia,  37 

( llossopharyngeus,  36 

( llossospagms,  67 

Glossj    skin.    13,    I  16.    216,    tso 

Glotzauge,  346 

( tlotzaugenkrankheit,  315 

Gluteal  point.  51 

Gluteal  reflex,  8 

1 1 ■ . •  r \  c  .  mi 

Goiter,  346 


472 


GENERAL    INDEX 


Goitre  exophthalmique,  3-15 

Goll,  column  of,  168 

Gordon's  symptom,  82 

Gothic  palate,  407 

Graefe'a  sign,  347 

Grand  ecart,  173 

Grand  hysteria,  444 

Grandiose  delusions,  204 

Graphospasms,  72 

Grave's  disease,  345 

Gray  communicating  branches,  370 

Gray  rami  communicantes,   13 

Gumma,  289 

Gummatous  meningitis,  216,  218 

Giirtelrose,  384 

Gymnastic  exercises,  55,  67,  197,  247 

Habit  pains,  438 

Habitus  apoplecticus,  264 

Half  cretins,  343 

Hammer  palsy,  3 

Hand  and  finger  centers,  275 

Hand  walker,  241 

Haphalgesia,  439 

Hard  traumatic  oedema,  382 

Hardness  of  hearing,  33,  35 

Harelip,  407 

Haut  mal,  389,  392 

Headache,  216,  223,  410,  454 

Headache  in  cerebellar  affections,  306 

in  purulent  cerebral  meningitis,  298 
Headache,  hysterical,  437 

neurasthenic,  410 
Headache  nodules,  459 
Heart  crises,  180 
Hederotopia,  165 
Heine-Media  disease,  235 
Helicopodia,  113,  260 
Hemato-ethyroidine,  356 
Hemialgia,  439 
Hemianesthesia,  440 
Hernia  nopsia,  26,  258,  458 
Hemiataxia,  308 
Hemiathetosis,  242 
Hemiballism,  80 
Hemichorea,  80,  242 
Hemicrania,  456 

cerebellar,  457 

continued,  457 

permanent,  457 

simple,  458 

simplex,  455 
Hemicranic  predisposition,  459 
Hemi-epilepsy,  391 
Hemiglossoplegia,  38 
Hemihypotonia,  308 
Hemiparaplegia,  157 
Hemiplegia,  7,  218 

atypical,  .'TO 

cortical,  270 

cruciata,  270 

extra  capsular,  270 

homolateral,  271 

lacunar,  271 

peduncular,  270 

pontine,  270 

sine  materia,  271 

spastica  infantilis,  330 

spinal,  157 
Hemming  in  of  affects,  450 


Hemorrhages  into  the  brain  substance,  328 

into  the  spinal  cord,  32(1 
Hemotomyelia,  149 
Hereditary  family  ataxia,  123 
Hereditary  predisposition,  407 
Heredosvphilis,  329,  333,  396 
Herpes,  250 

ophthalmic,  386 

zoster,  384 
Hip  flexion  phenomenon,  261 
Hippus,  81,  136,  183 
Hoffmann's  phenomena,  77 
Homochronous  heredity,  127 
Homolateral  hemiplegia,  271 
Homologous  heredity,  127 
Homosexuality,  444 
Hormone,  345 
Homer's  symptom  complex,  146,  149,  371,  372, 

376,  379 
Huntington's  disease,  85 
Hydatid  cyst,  289 
Hydrencephalocele,  321 
Hydrocephalic  cry,  249 
Hydrocephalic  idiocy,  337 
Hydrocephalus,  317,"  329 

external,  317 

ex  vacuo,  317 

internal,  317 

meningeal,  317 
Hydrocephaly,  317 
Hydrophobia,  68 

Hydrotherapy,  233,  355,  375,  435 
Hygiama,  268 
Hypacusis,  33 
Hypalgesia,  6 
Hypalgesia  dolorosa,  40 
Hyperacusis,  32 
Hyperalgesia,  6 

neurasthenia,  437 
Hyperaphrodisia,  421 
Hyperemisis  gravidarum,  4 
Hyperemia  of  the  brain,  304 
Hyperepinephria,  364 
Hyperesthesia,  6 

of  the  hairy  region,  438 
Hyperfunction  of  the  pituitary  gland,  368 
Hyperidrosis,  9,  349,  372 
Hyperkinesias,  65 
Hyperparathyroidosis,  89 
Hyperpituitarism,  366 
Hyperreflexion,  8,  113 
Hyperthyroidism,  353 
Hypertonia,  8,  113 
Hyperesthesia  dolorosa,  40 
Hypnalgias,  411 
Hypnosis,  452 
Hypoacusis,  34 
Hypochondria,  405 
Hypoesthesia,  6 
Hypoepinephria,  364 
Hypogenitalism,  368 
Hypogeusia,  30 
Hypoglossal  nerve,  38 
Hypoglossus,  38 

bilateral  paralysis,  38 
Hypoidrosis,  9 

Hypophyseal  eunuchism,  368 
Hypophyseal  insufficiency,  368 
Hypophysis,  367 

tumor,  366 


GENERAL    INDEX 


473 


Hyporeflexia,  8 
Hyposmia,  26 
Hypotaxia,  7 
1 l\  pothyreosis,  358 
Hypotonia,  8,  173,  308 
Hysteria,  137,  209,  43.; 
Hysterical,  anorexia,  4 1-1 

aphonia,  447 

arthralgia,  438 

attacks,  4  13 

aura,  444 

harking  cough,  445 

contractures,  448 

fever,  443 

hemorrhages,  443 

hyperthermia,  443 

longings,  411 

mutism,  447 

swollen  neck,  445 

paralyses,  446 

pemphigus,  443,  451 

pregnancy,  443 

psychoses,  451 

stigmata,  437 

ulceration,  443 
Hysterofrenatory  zones,  438 
Hysterogenic  zones,  439 
Hysteroneurasthenia,  405 

Ideational   apraxia,  206 
[deatory  apraxia,  284 
Ideo-kinetic,  285 

ataxia,  284 
Idco-motor  apraxia,  284 
[deomotor  pupillary  reaction,  183 
Idiocy,  327,  335,  336 
Idio-muscular  contraction,  350,  U3 
Idiopathic  facial  paralysis,  39 
Idiopathic  forms  of  spasms,  65 
Ileosacral  point,  51 
Imbecility,  336 
Impairment  of  speech,  279 
Imperativi  conceptions,  127 
Impotence,  286,  t-'l 
Incontinence,  intermittent,  155 

of  bowels,  I  Mi 

Of  urine.  ISfi 
Incontinence,  permanent,   l.jj 
Incoordination,  7,  123 
Increased    irritability   of  the  neuro-mnsenlar 

apparal  us.  413 
Indurative  headache,  158 

Infantile.   Cerebral    palsy,   330 

cerebral  pals}   without  paralysis.  333 
cerebral  paralj  sis,  ji  j 
multiple  sclerosis,  134 

muscular  at  rophy,  325 
Invxtcdcloa.  341,  359 

pareses,  201  ' 

progressive   hypertrophic   neuritis,    l:',l 
spastic    diplegia,    327 

spastic  hemiplegia,  327 

Infantilism.  337.  368,    107 

Inflai atory  atrophy,  27 

Inline,, /a,   303 

I  nfraoi  bital  point,  is 

Inhibition.    I  I!) 

Injection  therapy  in  facial  cramps.  117 
in    neuralgias.   60 

Innervation  apraxia,  885 


Instrumental  amusia,  2S3 
Intellectual  grand  mal,  393 

petit  mal,  393 
Intention  spasm,  76 
Intention  tremor,  134,  135,  311 
Intercostal  neuralgia,  50 
Intermittent    claudication    of    the    spinal    cord. 

226 
Intermittent  dropsy  of  the  joints,  382 
Intermittent  limping,  225,  373,  371 
Internal  secretion,  341,  345 
Internal  speech,  275 
Interparoxysmal  anomalies,  394 
Intestinal  crises,  181,  196 
Intoxication  tetany,  75 
Intracortical  hemiplegia,  270 
Intramedullary  tumors,   16] 
Intra-meningeal  hygroma,  317 
Intra-social  imbecility,  336 
Iodglidine,  193 
Iodide,  193 

intolerance,  221 
Iodine,  193 
Iodine  Basedow,  353 
Iodipin,  193 
Iodism,  221 
Iodon, 193 
Iodostarin,  193 

Iodotherapy,  193,  210.  321,  -'33 
Iodtropin,  193 
I ri tic  migraine,  458 
Irritable  breast,  50 
Irritable  testis,  53 
Irritable  weakness,  420 
Irritative   symptoms   on   the    part   of  the   sense 

organs,  411 
Irritative  weakness,  412 
Ischemic  disturbance  of  sensation,  373 
Ischuria  paradoxa,  137,  155 

Jacksonian  epilepsy,  218 

Jacksonian  epileptic  attacks,  293 

Jargon  aphasia,  278 

•law  spasms,  65 

Jendrdsoik'a  maneuver,   171 

Jiu-jitsu,  1  19 

Jughandle  cars,  394 

.1  umping  jack,  si 

.lumping  jack    limbs,  237 

Juvenile    paresis,    .'01 

Juvenile  scapulo  humeral  type,  98 

Kakke,  5 

Kalmuck  type  339 

ECeral ii is,  neuroparalytic,  5(> 

Kt  mig'a  sign,  249,  300 

ft  i  rnig't  sympi 198,  30  ' 

Kinesthetic  memorj    picture,  -'"3 

K  i, a, therapeutic  baths.  55 
Klumpke's  paralysis.  17,  117 
Koprolalia.  71 
Knbisagari,  93 

I  aciinte,  -'-'7 

lacunar  hemiplegia,  .'-'7.  jt i 

I  agopbthalmus.  32 

I  .aneinaling   pains.    ISO 

Landow  y  lh  j,  i  i,u   type  of  dystrophy,   109 
I, mill ni's  paralysis,  I-'.  159 
/,„„,   lannelongue  operation.  339 

I,iiiiiii 's  method,  60 


474 


GENERAL    INDEX 


Laryngism,  68 

tabetique,  185 
Larynx  crises,  180,  196 
Larynx  spasms,  68 
Lasagne's  sciatic  phenomenon,  51 
Late  epilepsy,  396 
Latent  heredity,  127 
Latent  tetany,  76 
Lateral  ganglion  system,  370 
Latero-pulsion,  88 
I.athyrism,  112,  213 
I.athyrus  cicera,  112 
Lathyrus  sativa,  112 
Laughing  spasm,  145 
Ivead  neuritis,  43 
Lead  paralysis,  43 

Lead  poisoning,  4,  39,  42,  72,  75,  112, 133, 158 
Leg  phenomenon,  77 
Leichens tern's  phenomenon,  249 
Leichens tern's  sign,  300 
Lepra,  4,  147,  410 
Lethargic  attacks,  445 
Letter  games,  282 

Lii/ihn-M  a  bins  type  of  dystrophy,  102 
Limb  kinetic  apraxia,  285 
Liniments,  58 
I.ipiodin,  193 

Lipodystrophy,  progressive,  387 
Lipoma,  289 
Literal  aphasia,  278 
Little's  birth  palsy,  332 
Little's  disease,  327 
Liver  crises,  181 
Lobar  sclerosis,  327,  329 
Local  asphyxia,  376 
Local  poisoning,  4 
Local  spasms,  65 
Local  syncope,  376 
Localized  muscular  spasm,  70 
Lockjaw,  65 
Loose  shoulder,  101 
Lordosis,  101 
Louise  Bryson's  sign,  349 
Lower  complexus  paralysis,  147 
Lues  cerebrospinalis,  131,  164 
Lues  nervosa,  165 
Lumbar  neuralgia,  50 
Lumbar  point,  51 
Lumbar  puncture,  293,  320 

technique  of,  253 

Macropsia,  389 

M  ml  el  nun's  disease,  387 

Mai  comitial,  388 

Mai  perforant,  179 

Malade  imaginaire,  405 

Maladie  des  tics,  71 

Malar  point,  48 

Malformations,  317 

Malleolar  point,  51 

Malleus  maleficarum,  441 

Malum  coxae  senilis,  53 

Mammary  point,  438 

Man-crazy,  436 

Mandibular   branch,   neuralgia   of,   47 

Mandibular  reflex,  28 

Marantic  confusion,  207 

Marantic  tabes,  179 

Marantic  thrombosis,  302 

Marginal  gliosis,  397 


Marinesco's  succulent  hand,  146 
Massage,  55,  60,  247 
Mast  cure,  71 
Masticatory  spasms,  65 
Mastodynia,  50 
Masturbation,  421 

Maternal   metrapectic   inheritance,  98 
Maxillary  neuralgia,  47 
Median  paralysis,  3,  19 

Median    peripheral    field    of    the    posterior   col- 
umn,  170 
Medullary  crises,  181 
Melancholia,   429 
Melanodermia,  363,  395 
Membres  de  polichinelle,  237 
Memoire  verbale,  272 
Mi  iiilil-Heehtereir  phenomenon,  114 
Mi  nilel-Bechterew  reflex,  136,  163,  261 
Miniiri's  attacks,  217 
M entire's  disease,  138 
Meningitis,  251 

cerebro-spinalis  siderans,  251 

of  the  convexity,  298 

purulent  cerebral,  298 

serous,  162 
Meningocele,  cranial,  321 

pedunculated,  321 

spinal,  322 
Meningococcic  endocarditis,  250 
Meningococcus  intracellularis,  248 
Meningococcus  sera,  252 
Meningo-encephalitis,  217 
Meningo-myelitis,  217 
Mental  point,  48 
Meralgia,  42 
Mercurial  tremor,  140 
Mercury,  193,  220,  334 
Metatarsal  point,  51 
Metatarsalgia,  53 
Micromania,  205 
Microcephalus,  317 
Microcephaly,  338 
Micro-encephaly,  338 
Microgyria,  329 
Micromelia,  341 
Micropsia,  389 
Migraine,  454 

equivalents,  459 

facioplegic,  458 

gustatory,  458 

of  the  arthritic,  458 

olfactory,  458 

ophthalmic,  458 

otic,  458 

prosoplegic,  458 

red,  456 

white,  456 
Miliary  aneurism,  264,  314 
Migrating  erysipelas,  160 
Millnril-Onbler  symptom-complex,  270 
Mimic  cramp,  66 
Mind  blindness,  286,  336 
Mind  deafness,  286,  336 
Minor  epileptic  attacks,  392 
Mixed  nerves,  24 
Maebius  sign,  347 
Moist  skin,  349 

Molimina  climacteria  virilia,  225 
Momentary  absence,  393 
Mongolism,  341 


GENERAL    INDEX 


475 


Mongoloid  idiocy,  339 
Monkey's  poliomyelitis,  2+3 
Monkey's  spinal  paralysis,  243 
Mononeuritides,  41 
Monoplegia,  7,  294 
brachialis,  236 

cruralis,  236  • 

facialis,  31 

facio  brachialis,  21S 

masticatoria,  30 
Morbo  di  Fiajani,  34.5 
Merlins  comitialis,  388 
Mori ms  sacer,  388,  393 
Morbus  vagabundus,  364 
Moria,  294 

Morning  paralysis,  241 
Morphinism,  388 
Mortal  depression,  409 
Morton's  disease,  53 
Morton's  metatarsalgia,  459 
Motility,  7 
Mntur  ataxia,  284 
Motor  points,  16 
Movable  heart,  41.5 
Mucous  colic,  116,  H3 

Multiple   necrotic  gangrene  of  the  skin,  377 
Multiple  sclerosis,   132,  142,  162,  2(19,  311 
Musei  volantes,  411 
Muscle  wave,  91 
Muscular  ankylosis,  113 
Muscular  atrophy,  97,  21.5 
Muscular  defects,  101 
Must  ular  madness,  61 
Muscular  spasms,  65 
Muscular  weakness  in  the  lower  extremities, 

3*8 
Muttering  delirium,  300 
Myalgic  migraine,  158 
Myasthenia,  93,   l  JO 
Myasthenic  reaction,  95 
Mydriasis,   is:; 
Myelitis,  1.59.  246 
Myelocystocele,  32:: 
.Myc.l,,  dysplasia,  321 
Myeloencephalitis,  disseminated,  140 
Myelomeningocele,  323 
Myoclonias,  73 
Myoclonus,  395 

epileps} .  '!'■'• 
Myokymia,  l<>.  II  t 
Myopathia,  primitive  progressive,  97 

rachitica,  9.' 
Myopathic  forms  of  muscular  atrophy,  93 
Myosis.   IS2.  371 
Mj  ospasia  com  ulsiva,  71 
Mythomania,  1 19 
Myotics,  is.' 
Myotonia,  90 

acquired,  91 

atrophic,  91 

congenital,  90 
Myotonic  reaction,  91 
Myotomv,  70 
Myxoede'ma,  353,  358 

congenital,  358 

infantile,     ;  .- 

operative,  36 1 

spontaneous,  358 

Nanocephaly ,  339 


Narcoleptic  attack,  393 
Nasal  crises,  181 

point,  48 
Natal   lesions,  32S 
Neck,  spasm  of  muscle  of,  68 
Neighborhood  action.  368 
Nerve  crural  paralysis,    31 

deafness,  185,  441 

mucus,  417 

suprascapular  paralysis,  17 

thoracic  paralysis,  17 
Nervi   nervorum,  41 
Nervosism,  105 
Nervosity,   10.5 
Nervous  erythism,  405 
Nervous  diarrhoea,  419 
Nervous  dyspepsia,  418 
Nervous  heart  weakness,    11.5 
Nervous  tachypnoea,   116 
Neural  form  of  progressive  muscular  atrophy, 

105 
Neuralgia,  45 

major,  48 

minor,  49 

nocturnal,  47 

of  the  feet,  53 

spermatic,  53 
Neuralgic  phenomena,  349 
Neurasthenia,  208,   107 

accidental,   422 

acquired,  122 

congenital,  422 

constitutional,  122 

cordis,  414 

sexual,  420 

true,   122 
Neurasthenic  arthralgia,   112 
Neurasthenic  helmet,  I  lo 
Neurectomy,  70 
Neurexaresis,  61,  198 
Neuritic  processes,  3 
Neuritides,  3 
Neuritis,  3,  40 

optic,  27,  217 

sciatic.  .52 
Neurogenic  degenerative  atrophy,  9 
Neurolytic  injections,  lil 

Neuromata,  5 

Neuromatosis.  .5 
Neuropathic  diathesis,  104,  407 
Neuropathic  heredity,  165 
Neurorecidiv,  386 
Neurorezidive,  220 
Neurotabes  peripherica,  193 
Neurotic  drops}  .  3SI 

Neurotomy,  * » I 
Neurol  rophy,  II.  320 

Nevralgie   des   cdentcs,    t" 

Newspaper  folder's  cramps,  72 

Nicotinism,  I-'.  115 

Nictitation,  66 

Nighl  terrors,  I  J6 

Nocturnal  epilepsy,  391 

Nodular  headache,   168 

Nonne-Apelt  reaction,  is; 

Normal  elect  mile  10 

Nuclei  of  the  posterior  column,  168 

Nj  ctalgias.    I  I  I 

\\  itagmus,  36,  I  'i.  135,  309 

Nvstagmiis-niyiicloiii.i.    I  ' 


47(3 


GENERAL    INDEX 


Object  agnosia,  286 
Obsessions,  427 
Occipital  neuralgia,  49 
Occupational  spasms,  72 
Oculo  motor  nerve,  27 

paralysis,  28 
(Edema  of  the  skin,  381 
CBsophagism,  (i8,  443 
(Esophagus  crises,  180 
(Esophagus  spasm,  68 
Olfactory   nerve,  2(i 
Olivo-ponto-cerebellar  atrophy,  315 
Olympic  forehead,  320 
Onanism,  421 
Onomatomania,  428 
Ophthalmic  migraine,  201,  457 
Ophthalmoplegia,  303 

chronica,  progressive,  121 

externa,  95,  :i-'l 

interna,  29,  218 

totalis,  31 
Oppenheim  reflex.  114,  134,  136,  163,  261,  300 
Optic  agnosia,  286 
Optic  aphasia,  280 
Optic  nerve,  26 

atrophy,  27,  184,  193 
Organotrophy,  44 
Osteoarthropathies,  146,  179 
Osteoma,  289 
Ovarian  point,  438 
Overuse  theory,  166 
Oxyakoia,  32 
Oxycephalus,  320 

Pachymeningitis,  hypertrophic  cervical,  148 

internal  hemorrhagic,  301 
Palanesthesia,  7 
Palate  reflex,  29 
Pallesthesia,  7 
Palmar  reflex,  8 
Palpebral  point,  48 
Palpitation,  415 
Panaris  analgt^siquc,  146 
Pantalgia,  439 
Papilledema,  290,  307 
Papillomacular  bundle,  184 
Paracusis  Willisii,  36 
Paradoxical   foot  phenomenon,  88 
Paragraphia,  279 
Parakinesia,  283 
Paralvsies  pareellaire,  184 
Paralysing  vertigo,  93 
Paralysis,  7,  184,  200 

agitans,  86 

ascendens  acutissima,  159 

ascending,  242 

bulbo-pontine,  242 

compression,  3 

diphtheritic,  43 

galloping,  206 

glosso-labio-pharyngeal,  118 

individual,  7 

Landry's,  242 

median,  3 

musculo-cuta  neons,  19 

muscular  spinal,  3,  20 

musculo  spiral,  2 

obstetrical,  3 

of  circumflex  nerve,  19 

of  long  thoracic  nerve,  17 


Paralysis  of  muscles  of  mastication,  324 

of  musculo-cutaneous  nerve,  19 

of  the  tongue,  324 

of  tibialis  posticus,  21 

paroxysmal,  93 

periodic,  93 

perineal,  2,  23 

pressure,  3 

professional,  2 

progressive,  184,  200 

progressive  bulbar,  116 

rheumatic  facial,  3 

serratus,  3 

sleep,  2 

spastic  spinal,  111 

spinal,  214,  219 

superior  bulbar,  121 

the  most  important  types,  16 

toxico-professional,  4,  43 

ulnar,  3 
Paralytic  face,  255 
Paralytic  pes  cavus,  239 
Paralytic  pulse,  292 
Paramyoclonus  multiplex,  73 
Paramyotonia,  91 
Paraphasia,  272,  279 
Paraplegia,  7 
Paraplegic  paralysis,  332 
Paraplegic   rigidity,  334 
Parapraxia,  284 
Parasitic  cysts,  289 
Parasvphilis,  164 
Parectropia,  283 
Paresis.  7,  200 

circular  form,  206 

professional,  3 
Paresthesias,  412 
Paresthetic  neuralgia,  42 
Paretic  seizures,  204 
Parietal  point,  48 
Parkinson's  disease,  86 

without  agitation,  88 
Paroxysmal   gastroxynsis,  416 
Partial  pseudo-hypertrophy;  99 
Partial  R.  D.,  11 
Passionate  attitudes,  445 
Patellar,  clonus,  114 

reflex,  8 
Pathformation,  55 
Pathographies,  395 
Pathomimicry,  451 
Peau  moite,  349 
Peetoralis,  defect  of,  325 
Peduncular  hemiplegia,  270 
Pellagra,  112,  213,  364 
Pension  hysteria,  447 
Periodic  oculomotor  paralysis,  458 
Peripheral  nerves,  1,  13 

diseases  of,  26,  145 

neuritides,  41 
Peripheral   pseudo-tabes,   191 
Permanent  drainage  of  the  ventricle,  321 
Peroneal  point,  51 
Perseveration,  284 
Pes  calcaneus,  146,  239 
Pes  equino-varus,  23,  146 
Pes  valgus,  239 
Petit  mal,  392 
Phantasy,  449 
Pharyngism,  68,  443 


GENERAL    INDEX 


477 


Pharynx,  crises,  180 

spasms  of  muscle  of,  68 
Phoenix,  57 
Phosphate,  355 
Phosphaturia,  416 
Phrenic  nerve  neuralgia,  49 
Pianoplaver's  cramps,  72 
Pica,  44l" 

Pied  tabetique,  179 
Pill  rolling,  87 
Pinpoint   pupils,   183 
Pithiatism,  440 
Plain  neurasthenias,  414 
Plantar  reflex,  8 
Platysma  phenomenon,  261 
Plegias.  ? 
Plagiocephaly,  336 
Plexiform  neuroma,  5 
Plexus  neuritides,  10,  41 
Pluriglandular  insufficiency,  368 
Polioencephalitis,  242 

inferior,  121 

inferior,  hemorrhagica,  303 

superior,   121 

superior  hamorrhagiea,  303 
Poliomyelitis,   anterior,   157 

anterior  acuta  infantum,  235 

chronic,  109 
Pollutions,    121 

Polydipsia,  443 

Polymorphous  tremor,  442.  443 
Polymyositis,  interstitial,  379 

Polyneuritic  psychosis,  10 1 
Polyneuritides,  11 
Polyneuritis,  10,  13,  40,  41,  246 
Polyopia,  monocular,  441 
Pontine  hemiplegia,  270 
PooISchlesingi  r  sign,  77 
Popliteal  point,  51 
Porencephaly,  327 

prenatal.  328 

primary.  327 
Poriamania,  394 
Positive  Rinnr.  31 
Postdiphtheritic  ataxia,  43 
Posthemiplegic  chorea,  80 
Posticus  paralysis,  37 

Posterior  horn   type-  of  sensory  anomaly,  145 
Posterior  perforating  point,  50 
Posterior  radicotomy,  163 
Posterior   root,.   166 

syndrome,  172 
Poir.t  disease,  163 
Preacher's  hand,  1 16,  148 
precordial  anxiety,  415 
Prehemiplegic  chorea,  80 
Prenatal  lesions,  327 
Premal  ure  senescence,  I  -'s 
Pressure,  paralysis,  162  , 

pulse,  -M|i 
Priapism,  156 
Procursive  epilepsy,  398 
Procursive  epileptic  attack,  393 
Prognathism,  kit 

Progressive  locomotor  ataxia,  87,  164,  182,  200 
Progressive  muscular  atrophy,  97 
Progressive  muscular  dj  trophy.  97 
Progressive  primitive  myopathy,  97 
Progressive  spinal  muscular  atrophy.  120,  117 
Pronation  phenomenon,  11a 


Propulsion,  88 
Prosoplegia.  31 
Protargol,  194 
Protean  neurosis.  US 
Provocative  agents,  47 
Pruritus,  nervous,  112 
Psammoma,  289 
Pseudo-ankle  clonus,   413,   447 
Pseudo,  bulbar  paralysis,  120,  229 

hypertrophy,  98 

membranous  colitis,  417 

microcephaly,  :i:(s 

paralysis.  209 

parencephaly,  327,  329,  337 

tabes.  165 

tumor,  161 

tumor  cerebri,  288 
Psychalgia,  439 
Psychasthenic   HIS,  428 
Psychic  anomalies,  409 
Psychic  aura,  389 
Psychic  changes,  349 
Psychic  equivalents.  11.5 
Psychic  powerlessness,  128 
Psycho-analysis,  150 
Psychographic  disturbances,  429 
Psychoneuroses,  103,  105 
Psychopathia  sexualis,  ill 
Psychotherapy,  70,  73,  199,  232,  429,  452 
Ptosis,  28,  94 

hysterical,    II? 
Pulmonary  osteoarthropathy,  367 
Pulsus  inaequalis,  tl."> 
Pulsus  respiratione  intermittens,  415 
Pupillary  alterations.  202 
Pupillary  functions,  is." 
Pupillary  rigidity.   183,  -'02.  217 
Pupillary  symptoms,  182 
Pupils,  sluggishness  of,  183 
Pure  alexia,  280 
Pure  word   deafness,    878 
Pure  word  dumbness,  277 
Puzzling   trophoneurosis,   451 
Pyelo-nephritis,  1S9 
Pyknosis,  -'I  I 

Pyramidal  tracts,  physiology  of,  112 
Pyramids,  decussation  of,  112 

Quinine  therapy,  3?  I 
Quinke'i  oedema,  381,  382 
Quinquaud't  phenomenon,  <>'i 
Quinquaud's  symptom,  ill 

Rachialgia,  II.' 

Rachischisis,  32! 

Rachitic  skull.  319 

Radial  phenomenon,  163 

Radicotomy,  163 

Radiculalgia,  l"> 

Radicular  neuralgia.  I '. 

Radicular   type  of  disturbances  of  sensibility, 

17.) 
Radiculitides,  II 
Radius,  reflex,  s 

Kami  communicantes,  alia.  370 

irrisei.  370 
Raynaud'*  disease,  375,  377 

Ri  ich f  (l.  gener  itlon,  10,  1 1,  .'37 

Hecklinghaun  n't  disea  •  .   I 
Recovery  « iih  defect,  219 


478 


GENERAL    INDEX 


Rectal  crises,  181 . 
Red  migraine,  460 
Reflex  epilepsy,  396,  401 
Reflex  neuralgias,  45 
Reflexes,  7 
Regeneration,  6,  9 
Regionary  cyanosis,  376 
Reinversion,  233 
Renal  crises,  181 
Reserve  centers,  273 
Residence  in  high  valleys,  354 
Respiration  fatigue,  225 
Respiratory  disturbances,  414 
Respiratory  spasms,  445 
Retention  of  urine,  186 
Retrobulbar  neuritis,  27 
Retrocollis,  69,  448 
Retrograde  amnesia,  305 
Hetropulsion,  88 
Retropulsive  epilepsy,  393 
Revulsives,  60 

Rhizotomia,  posterior,  116,  335 
Rhomboid  spasm,  69 
Rinne's  positive,  34 
Rhine's  test,  34 
Risus  sardonicus,  66 
Robertson's  symptom,  182 
Rodagen,  356 
Romberg's  disease,  383 
Romberg's  phenomenon,  173 
Root,  neuralgia,  45 

neuritides,  41,  45 

pains,  163,  295 
Rotary  spasms,  69 
Rotary  vertigo,  309 
Rushes  of  blood,  416 
Rust's  phenomenon,  163 

Sadism,  444 

Sajodin,  193 

Salaam  spasm,  69,  392 

Saltatory  reflex  spasm,  71 

Salt  free  diet,  399 

poor  diet,  399 
Salvarsan,  160,  193,  211,  220 
Salvarsanized  serum,  212 
Sandmannli,  344 
Sandwybli,  344 
Sarcoma,  288 

Saturnism,  158,  329,  388,  396 
Sawyer's  cramps,  72 
Scapula  alata,  17,  395 
Scapular  reflex,  8 
Scapulo-humeral  reflex,  8 
Scapulopesia,   105 

Schleich's  infiltration  anesthesia,  60 
Srhloesser's  injection,  61 
Schultze's  comma,  170 
Schurbach'a  test,  34 
Schwann's  sheath,  5 
Sciatica,  51 

varicose,  51 

varicose,  spasmodic,  52 
Sciatic  scoliosis,  52 
Scintillating  scotoma,  458 
Sclerodactylism,  378 

annular,  378 
Scleroderma,  377 
Sclerodermic  en  bandes,  379 
Sclerodermic  en  coup  de  sabre,  379 


Sclerose  en  plaques,  132 
Sclerosis,  139,  239 

amytrophic,  140 

bulbar,  139 

hemiparetic,  139 

multiple,  121,  132,  142,  162 

paraplegic,  139 
Scotoma,  26 

Screaming  spasm,  71,  445 
Scrotal  tongue,  340 
Seamstress's  cramps,  72 
Secondary  degeneration,  157 
Secondary  porencephalies,  327 
Seelig-Mueller's  neuralgia,  47,  127 
Segment  innervation,  152 
Self-perception,  411 
Senile  incontinence,  230 
Senile  spastic  paraplegia,  227 
Sensation,  disturbances  of,  25 
Sense  impressionability,  449,  453 
Sense  of  cold,  6 

of  heat,  6 

of  movement,  6 

of  pain,  6 

of  position,  6,  7 

of  pressure,  6 

of  taste   (testing),  32 

of  temperature,  6 

of  touch,  6 
Sensibility,  6 

disturbances  of,  175 

of  deep,  175 
of  superficial,  175 
Sensitive  crossway,  257 
Sensory  aphasia,  272 
Serous  apoplexy,  271 
Serous  meningitis,  288 
Serous  meningitis  of  the  posterior  fossa  of  the 

skull,  313 
Severe  epileptic  convulsive  attack,  389 
Sexual  neurasthenia,  409 
Shaking  disease,  442 
Shaving  cramp,  72 
Shock,  149 

Shoemaker's  cramp,  72 
Shoulder,  spasms  of  muscle  of,  68 
Siderodromophobia,  427 
Signe  de  peaucier,  Jul 
Signs  of  overirritabilitv,  349 
Silver  nitrate,  193 
Simple  atrophy,  27 
Singultus,  71 
Situation  anxiety,  427 
Skeletal  muscles,  disturbances  of,  185 
Skeleton  hand,   108,   118 
Skin  reflexes,  8 
Sky-high  exaltation,  409 
Sleep  inversion,  223,  233 
Sleeping  sickness,  209 
Sleeplessness,  410 
Sleeplessness  drugs,  210,  233 
Smith's  cramps,  72 
Snake  man,  173 
Sneezing  reflex,  29 
Sneezing  spasm,  71 
Snoring  reflex,  71 
Softening  of  the  brain,  200 
Softening  of  the  cortical  layer,  200 
Solitary  tubercle,  289 
Somnambulism,  445 


GENERAL    INDEX 


479 


Spanish  boots,  180 
Spasm  nodding,  69 
Spasm  of  glottis,   76 

of  splenitis,  69 
Spasmodic  epileptiform  neuralgia,  -18 
Spasmophilia,  78 
Spastic  spinal  infantile  paraplegia,  332 

spinal  paralysis,  111,  115,  226 

symptom-complex,  112,  lit 
Spasticity  of  the  limbs,  337 
Speech  disturbance,  202 
Spermatorrhoea,  1.' 1 
Sphincter  disturbances,  186 
Sphinx  countenance,  101 
Spina  bifida,  322 

occult,  323 
Spinal  ectopies,  321 
Spinal  epilepsy,  65 
Spinal  fluid  phenomenon,  307 
Spinal  foci   of  disease,  32H 
Spinal   form  of  muscular  atrophy,  107 
Spinal  ganglia,  166 
Spinal   gliosis,  143 
Spinal  heredo-ataxia,  123 
Spinal  intermittent  limping,  115,  215 
Spinal  irritations,  405,  412 
Spinal  progressive  muscular  atrophy,  245 
Spirochete,  21 1 

Spontaneous  fractures,  178,  239 
Spontaneous  gangrene,  377 
Spontaneous  recoveries,  319 
Sporadic  cretinism,  358 
Stage  of  local  reddening,  376 
Stage  of  reaction,  263 

Stasobasophobia,  117 

Status,  choreicus,  81 

criticus,  isit 

crihrosus,  227 

epileptieus,  180,  208,391 

hemicranicus,  457 

hydroceplialicus,  251 

lacunaris,  227 
St,  cple  skull.  320 
Stellwag's  sign,  317 

Steppage,  23 

Stereo-anesthesia,  7,  177 

Sternal   point,    138 
Stiff   neck.  249 

Stiffness  of  the  neck,  ?98 

Stigmata  of  degeneration,  165,  336,  394,  107, 

m 

Stork  legs,  106 

StrHmpelPs  sign,  115 

Struggle  for  pension,  1  !5 

Struma  suprarenalis,  363 

Strychnine,  194 

Stuttering,  \S6 

Subacute  anterior  poliomyelitis,  245 

Subarteria]  spinal  tracts,  112  , 

Subcortical  sensory  aphasia,  !78 

Supiti  itor  longus,   1 1 

Superficial  sensibilities,  7 

Supporting  corset,  ins 

Supportive  apparatus,  116,  198 

Supraorbital  neuralgia,  11 

Supraorbital  point,  is 

Suspension  treatment,  197 

Surgery  of  tlie  central  convolutions.  ."I  I 
Surprise  method,  152 
Sweat  secretion,  9 


Sydenham's  chorea,  80 
Syllable  tumbling,  202 
Symmetric  lipomatosis,  387 
Sympathetic,  crises,  180 

diseases  of  the,  369 

nervous   system,  369 

ptosis,  147,  371 
Symptom  of  approximate  answers,  445 
Symptom  of  painful  thinking,  222 
Symptomatological  triad,  139 
Syncopal  attacks,   115 
Syncope,  304 
Syndactylism,  395 
Synergic  pupillary  reaction,  183 
Syphilis,   158,  164,  311,  317,  425 

a  virus  nerveux,  165,  192 
Syphilitic,  endarteritis  obliterans,   361 

spinal  amyotrophy,  186 
Syphilogenic  diseases,  nil.  tsj,  200,  213 
Syphilogenic   combined   system    diseases,   213 
Syringo  bnlbia.  143,  147 
Syringomyelia,  4,  120,  143 
System  diseases,   111,  166,  213 
Systematic  vertigo,  309 


Tabes,  acuticima,  190 

arrested  by  blindness,  189 

cervical,  190 

dorsalis,  164,  182 

dorsaiis  spasmodique,  111,  121 

inferior,   190 

paralysis,  189 

rudimentary,  190 

superior,   KM) 

visceral,  190 

without  spinal  cord  symptoms,  190 
Tabetic   foot,   179 
Taboparah  sis.  201 
Tache  cerebrate,  209,  250 
Tachycardia,  37,  317 

paroxysmal,   41  t 
Tachynoic  attacks,  445 

'1'actilc-   agnosia.   287 
Tactile  aphasia.  .'SO 
Tailor's  cramps,  12 
Talalgia,  53 
Talipes  calcaneus,  21 

Tapir  snout,    101 
Tarsalgia,  53 
Tartar  type,  339 

Tay'i  cherry  red  spot,  :t:ts 

7,ii/  Sack's   disease, 

Teichopsia,  157 
Telegrapher's  cramps,  72 
Telephone  operators.  2 
T,  mperat  ure  sense.  6 

Temporal    point.    Is 

Temporary   infantile  spinal  paralysis.  241 

Tendinitis,   calcareous.  379 

Tendon  reflex,  s 

(ran    plantation,    107 

Tenotomy,  105,  107,  116 
Teratoma,    189 

Terminal   delirium,    115 
Testicular  crises.  1S1 

Tetany,  ?i 

Tetraplegia.  7,    !Mi 

The   man    with    (be   litlle    paper    .    ins 

Therapy  of  litil,-  symptoms,  ft." 


480 


GENERAL    INDEX 


Thermoanesthesia,  6 

Thermohypoesthesia,  6 

Thermotherapy,  58 

Thomsen's  disease,  90 

Throat,  spasm  of  muscles  of,  68 

Thrombosis  of  basilar  artery,  121 

of  the  brain  sinuses,  302 
Thyradine,  361 
Thyreoaplasia,  341,  358 
Thyrogenous  theory  of  Basedow's  disease, 

322 
Thyroidism,  362 
Tibial  phenomenon,  115 
Tic,  73,  428,  443 
Tigroid  scales,  244 
Tigrolysis,  244 
Tinnitus  aurium,  309 
Toe  phenomena,  114 
Tongue,  spasm  of,  67 
Tonic  reflex,  82 
Tooth  spasms,  388 
Topalgias,  438 
Torpor  peristalticus,  419 
Torsion  neurosis,  86 
Torticollis,  448 
Touch  paralysis,  295 
Touch  sense,  6 
Tachycardia,  347 
Tracts  of  spinal  cord,  158 
Tracts  which  degenerate  downward,  158 
Tracts  which   degenerate   upward,   158 
Tractus,  spino-cerebellar,  169 

spino-thalmicus,   167 
Tragic  look,  346 
Transcortical  aphasia,  279 
Transference,  439 
Transplantation,  248 
Transposition  of  motor  points,  16 
Transverse,  laughing,  102,  119 

lesions  of  the  spinal  cord,  150 
Traumatic  hysterics,  442 
Traumatic  neuritis,  6 
Traumatic  neurosis,  424 
Treatment  in  sanitariums,  431 
Tremor,  63,  203,  414 
hysterical,  414 
in  Basedow's  disease,  348 
neurasthenic,  414 
of. the  degenerate,  64 
vibrating,  63 
Trepidation,  epileptoide,  114 
Triceps  reflex,  8 
Trigeminal  neuralgia,  47 
Trigeminus,  29 
Triplegia,  7,  236 
Trismus,  65 
Troehleares,  29 
Trophic  disturbances,  350 
Trophic  functions,  9 
Trophoedeme,  familial,  381 
Trophoneuroses,  369 
Trousseau  sign,  77 
True  plethora.  304 
Trumpeter's  cramps,  72 
Tubercula  dolorosa,  5 
Tubercles  in  the  chorioid,  300 
Tuberculous  meningitis,  299 
Tuberous  sclerosis,  328,  329 
Tumors  in  the  region  of  the  corpora  quad- 
rigemina,  368 


Tumors  of  basis  of  brain,  294 

of  cerebello-pontine   angle,  288,  239,  311 

of  frontal  lobe,  294 

of  occipital  lobe,  295 

of  region  of  the  optic  thalamus,  294 

of  spinal  cord,  160 

of  temporal  lobe,  295 

of  vermis,  309 

Urinary  stuttering,  421 
Urticaria,  381 

Vaginismus,  421,  443 
Vagotony,  371 
Vagus  crises,   180 
Vagus  nerve,  37 
Vagus  paralysis,  37 
Valerian  saturation,  355 
Valleix  points,  46,  457 
Vapors,  405 
Vasa  corona,  243 
Vasoconstriction,  9 

Vasoconstrictor  neuroses  of  the  extremities,  373 
"Vasodilators,  9 
Vasodilator  neurosis,  382 
Vasomotor,  angina  pectoris,  456 
functions,  9 
trophic  neuroses,  372 

Vegetative  nervous  system,  369 

Veitstanz,  80 

Ventral  field  of  the  posterior  column,  170 

A'entricles,  puncture  of.  321 

Verbal  amnesia,  279 

Verbal   paragraphia,   278 

Verbal  paraphasia,  277 

Vermicular  response,  11 

Vertebral  column,  affections  of,  162 

Vertebral  point,  50 

Vertical  divergence  of  the  eyeballs   (of  Magen- 
die),  310 

Vertigo,  411 
permanent,  412 

Vesical  crises,  181 

Vesico  spinal   center,  155 

Vestibular  attacks,  309 

Vestibular   nerve,   33 

Vigouroux's  phenomenon,  349 

Visceral  nervous  apparatus,  369 

Vitiligo,  387 

Vltlpian's  conjugate  deviation,  262 

Waddling  gait,  103 
Waller's  law,  5,  8,  157 
Wandertrieb,  394 
Was])  waste,  102 
Wassermann   reaction,  164,  221 
Watchmaker's  cramps,  72 
Weakness  of  memory,  209 
Weber's  symptom-complex,  270 
Weber's  test,  35 
Weeping  spasm,  445 
Weir-Mitchell  rest   cure,  435 
Werding-Hoffmann  type  of  progressive  muscu- 
lar atrophy,   107 
Wernicke's  aphasia,  276 
Wernicke's  zone,  272 
West/thai'.*  phenomenon,  174 
Westphal's  pseudo-sclerosis,  141,  187 


GENERAL    INDEX 


■481 


White  communicating;  branches,  870 

White  migraine,  460 

Witch  signs,  441 

Witzelsucht,  394 

Word  deafness,   276 

Word  debris,  2~1 

Word  dumbness,  276 

Word  memory.  272 

Wrist  clonus,  114 

Wri^t  drop,  20,  54 

Writer's  cramps,  ~2 


Xantho  chromia,  \t>2 
Xylographers,  2  ' 
Xyrospasms,  72 

Yawning  spasms,  71 

Zither  player's  cramps,  72 
Zona,  384* 

Zona  cornu-commissularis,   170 
Zona  meduUo-vasculosa,  8S3 
Zona  septo-marginalis,  170 


i  -0/*"^ 


COLUMBIA   UNIVERSITY 

This  book  is  due  on  the  date  indicated  below,  or  at  the 
expiration  of  a  definite  period  after  the  date  of  borrowing, 
as  provided  by  the  rules  of  the  Library  or  by  special  ar- 
rangement with  the  Librarian  in  charge. 

DATE  BORROWED 

DATE  DUE 

DATE  BORROWED 

DATE  DUE 

njC 

MAY  2 

\  1945 

" 

tfjUV 

rMil 

f 

WW 

9  ?.  A**1  . 

\&h 

]AY3i194^ 

«a,e3a,M=o 

